IMPROVING ADENOVIRUS-BASED VACCINE EFFICACY BY THE INNATE IMMUNE MODULATORS EAT-2 AND REA By Yasser Ali Aldhamen A DISSERTATION Submitted to Michigan State University in partial fulfillment of the requirements for the degree of DOCTOR OF PHILOSOPHY Microbiology and Molecular Genetics 2012 ABSTRACT IMPROVING ADENOVIRUS-BASED VACCINE EFFICACY BY THE INNATE IMMUNE MODULATORS EAT-2 AND REA By Yasser Ali Aldhamen Despite recent advances, there is a great need for more potent formulations to enhance immunogenicity of vaccines. Pro-active induction and/ or harnessing of beneficial innate immune responses may be the mechanism underlying the effectiveness of certain adjuvants to significantly contribute to the ability of vaccines to generate adaptive immune responses. Several classes of immune receptors have been targeted for novel adjuvant development. An important emerging class of immune receptors is the SLAM family of receptors. We endeavored to develop a strategy to improve the efficacy of vaccines by incorporation of proteins known to be important in SLAM mediated signaling. In this dissertation, we described that coexpression of the SLAMꞌs adaptor, EAT-2, along with pathogen derived antigens facilitates induction of beneficial innate immune responses, resulting in improved induction of antigen-specific adaptive immune responses. We utilized recombinant Adenovirus-based vaccines expressing murine EAT-2 along with HIV-1/Gag or Plasmodium falciparum derived circumsporozoite (CS) protein. As compared to appropriate controls, rAd5 vectors expressing EAT-2 facilitated bystander activation of NK-, NKT-, B-, and T-cells early after their administration into animals. EAT-2 expression also augments the expression of surface maturation markers of antigen presenting cells (CD40, CD80, CD86, MHC-II, and CCR7). Indeed, this multi-tiered activation of the innate immune system by vaccine mediated EAT-2 expression enhanced the induction of antigen specific cellular immune responses. We also compared the utility of vaccine mediated ii expression of EAT-2 relative to another innate immune stimulator, a recombinant TLR agonist derived from Eimeria tenella, referred to as rEA. We confirmed that rEA activates multiple immune cell types and elicits the induction of pleiotropic pro-inflammatory cytokines in a MyD88-dependent manner. Surprisingly, we also found that the TRIF adaptor protein acts as a potent negative regulator of TLR agonist-triggered immune responses. However, we uncovered a potent immunosuppressive activity inherent to the combined expression of the CS protein and rEA, in contrast to continued enhancement of CS specific adaptive immune responses by use of the EAT-2 adjuvant. We subsequently went on to partially unveil the mechanism underlying the ability of the EAT-2 adaptor protein to regulate the induction of adaptive immune responses. We demonstrate that EAT-2 expression specifically prevents vaccine induced CRACC upregulation on APCs in a MAPK-dependent mechanism. Confirming these results, utilization of a mutated SH-2 domain form of EAT-2 adaptor (EAT-2(R31Q) failed to prevent CRACC upregulation. We have also demonstrated that EAT-2 expression triggers the production of several cytokines and chemokines from macrophages in a MAPK-dependent and independent mechanisms. Future studies will further expand upon these findings. We leave our readers with the view that, understanding the molecular mechanism and the role of SLAM/ EAT-2 and rEA adjuvant activity, will allow for the development of next generation vaccine platforms for use in a number of immunotherapy approaches targeting HIV-1 and Malaria derived antigens, as well as numerous other vaccine targets in general. iii DEDICATION I dedicate this dissertation to my wonderful family. Particularly to my understanding and patient wife, Suha, who has put up with these many years of research and to our precious daughter Seba and son Ahmed, who are the joy of our lives. Also, I would like to dedicate this doctoral dissertation to my parents. There is no doubt in my mind that without their continued support and counsel I could not have completed this process. iv ACKNOWLEDGEMENTS I would like to gratefully and sincerely thank my mentor Dr. Andrea Amalfitano for his guidance, understanding, patience, and most importantly, his friendship during my graduate studies at Michigan State University. I have been amazingly fortunate to have an advisor who gave me the freedom to explore on my own, Dr. Amalfitano constantly supported and encouraged me to develop scientific thinking, writing skills, critical data analysis and planning of experiments. I am not sure many graduate students are given the opportunity to develop their own individuality and self-sufficiency by being allowed to work with such independence. I specifically thank Dr. Amalfitano for supporting and supervising the rAd5-EAT2 project, a project that completely transformed my research here at Michigan State University. For everything you’ve done for me, Dr. Amalfitano, I thank you. I hope that one day I would become as good an advisor to my students as Dr. Amalfitano has been to me. I greatly appreciate the help provided by my guidance committee: Dr. Norbert Kaminski, Dr. Sungjin Kim, Dr. Kefei Yu, and Dr. Ian York. Their insightful comments and constructive discussions have significantly improved my research progress as well as development of critical scientific thinking. I also thank my friend and co-worker, Dr. Sergey Seregin, who has been always there to listen and give advice. Sergey and I performed tremendous amount of experiments working with our novel Ad vectors, Ad5-DAF and Ad5-EAT2. I am deeply grateful to him for the long discussions that helped me sort out the technical details of my work. I am also thankful to him for encouraging and helping me to build Ad5-EAT2 vaccine vector. I also thank Dr. Daniel Appledorn. Dr Appledorn's insightful comments and constructive criticisms at different stages of my research were thought-provoking and they helped me focus v my ideas. I am grateful to him for helping and supporting my research as well enforcing strict validations for each research result, and thus teaching me how to do research. My sincere thanks to all current and former members of Dr. Amalfitano lab: Dr. Charles Aylsworth, Sarah Godbehere Roosa, Nathaniel Schuldt, Aaron McBride, Tyler Voss, Joyce Liu, Youssef Kousa, Dionisia Quiroga, David Rastall, William Nance, William De Pas, Megan Hoban, Jenny Zehnder, Brandy Burke. All of them are genuinely nice and eager to help each other, and I’m glad, I have worked and interacted with them. My special thanks to Sarah Godbehere Roosa, who is a wonderful technician and exceptionally pleasant person to work with. Special thanks to all MSU core facilities, including: ULAR and, in particular, all employees of BPS/biochemistry animal housing facility; MSU Histopathology labs; MSU Genomics Technology Support Facility: Flow Cytometry (Dr. Louis King), quantitative RT-PCR (Jeff Landgraf). Without their great performance and skilled assistance our research would not have been possible. I’m thankful to all faculty members and employees of the Department of Microbiology and Molecular Genetics, especially the Chair of the department (Dr. Walter J. Esselman) and the Director of MMG Graduate program (Dr. Robert Hausinger) for their kindness and full support provided during these years. Most importantly, none of this would have been possible without the love and patience of my family. My family, to whom this dissertation is dedicated to, has been a constant source of love, concern, support and strength all these years. I would like to express my heart-felt gratitude to my family. My family has aided and encouraged me throughout this endeavor. I warmly vi appreciate the generosity and understanding of my family. Finally, I appreciate the financial support from The Ministry of Higher Education of Saudi Arabia, King Abdullah Bin Abdulaziz Scholarship Program. vii TABLE OF CONTENTS List of Tables……………………………………………………………….…....xvi List of figures……………………………………………………………………xvii List of Abbreviations………………………………………………………….....xxi Chapter 1 1.1 1.2 1.2.1 1.2.2 1.3 1.3.1 1.3.2 1.4 1.5 1.6 1.6.1 1.6.2 1.7 1.7.1 1.7.2 Chapter 2 2.1 2.1.1 2.1.2 2.1.3 2.1.3.1 2.1.3.2 Introduction…………………………………………...…………1 Innate immune system…….…………………….…………...…..2 Adaptive immunity and immunological memory …………….....4 T cells activation and cellular immunity...…………................…6 B cell activation and humoral immunity……………….………..12 Regulation of adaptive immunity by the innate immune system……………………………………………………………15 Innate immune cell-dependent regulation of the adaptive immune system…………………………………………………..16 The impact of innate immune recognition on adaptive immune cell responses………………………………………………….....21 Vaccine Adjuvants………………………………………………23 The immuno-modulatory molecule: recombinant Eimeria tenella derived antigen (rEA……………………………………..24 Harnessing innate immunity by the SLAM family of receptors adaptor EAT-2…………………………………………………...26 The signaling lymphocytic activation molecules (SLAM) family ofreceptors………………………………………………..26 Signal transduction of SLAM family of receptors by SAP family of adaptors………………………………………………..28 Adenoviruses as vaccine vectors………………………………....31 Molecular basis for cellular recognition of Adenovirus Vectors……………………………………………………………33 Adaptive immune responses to Ad vector expressed transgenes………………………………………………………..35 Expression of the SLAM family of receptors adaptor EAT-2 as a novel strategy for enhancing beneficial immune responses to vaccine antigen………………………………..........37 Introduction……………………………………………………...38 Human immunodeficiency virus (HIV) and AIDS global epidemic…………………………………………………..39 HIV genetic diversity and its impact for HIV control…………...40 Immune response to HIV-1 and immune evasion mechanisms….41 Innate immune responses to HIV-1……………………………...41 Adaptive immune responses to HIV-1…………………………..45 viii 2.1.4 2.2 2.3 HIV-1 vaccine Studies………………………………………..…48 Results…………………………………………….……..……...55 Discussion…………………………………………….……..…..89 Chapter 3 Vaccine platforms combining Circumsporozoite protein and potent immune modulators, rEA or EAT-2, paradoxically result in opposing immune responses …………………..…….…93 Introduction……………………………………………………...94 Immune responses to Plasmodium parasites…………………….95 Malaria vaccine development……………………………………97 Results …………………………………………………………..103 Discussion…………………..…………………………………...134 3.1 3.1.1 3.1.2 3.2 3.3 Chapter 4 4.1 4.2 4.3 Chapter 5 5.1 5.2 5.3 Chapter 6 6.1 6.1.1 6.1.2 6.1.3 6.1.4 6.2 6.2.1 6.2.2 6.3 6.4 6.5 6.6 6.7 6.8 6.9 6.10 6.11 TRIF is a critical negative regulator of TLR agonist mediated activation of dendritic cells in vivo …….………………..…..…..140 Introduction…………………….………………………….…….141 Results…………………………………………………………...144 Discussion……………………………………………………….165 Preventing CRACC receptor upregulation in antigen presenting cells improves induction of antigen specific adaptive immune responses by vaccines.……….……… ………………………….173 Introduction…………...…………………………………………174 Results………………....…………………………………………177 Discussion………………….…………………………………….200 Material and Methods…………………………..………………....207 Adenovirus vector construction…………………… ……….........208 EAT-2 expressing Ads construction……………..….……..……..208 Ad-HIV/Gag construction…………………………..……………209 Ad-CSP construction……………………………….…….............209 Ad-GFP and Ad-GFP/rEA construction……………..….……….209 Validation of viral particles (VP) titers of Ads………..………....210 Silver Staining…………………………………………..………..210 Western Blotting………………….…………………..…….........210 Animal procedures……………………….………….…….……..211 Cytokine and chemokine analysis……………………..…………212 Quantitative RT-PCR Analysis………………………..…………212 Isolation of Splenocytes…………………..……………..……….214 Cell staining and flow cytometry………………………..……….215 In vitro cell culture………………………………………..……....216 Murine CD11c+ DCs isolation……………………………..……..217 Murine IL12p70 measurement by ELISA from isolated CD11c+ DCs……………………………………………......…….217 CD8+ T cells depletion Analysis………………….……………....218 ix 6.12 6.13 6.14 6.15 6.16 ELISPOT Analysis………………………………………………..218 In vivo CTL Assay………………………………………………...219 Detection of CSP antibody in murine serum by ELISA…….....….220 Western blotting…………………………………………………...220 Statistical analysis………………………………………………....221 Chapter 7 Overall summary and significance ………………………………..223 Bibliography………………………………………………………………..……..230 x LIST OF TABLES Table 1 Table 1: Expression pattern of SLAM family of receptors in hemopoietic cells………...……………………………………..27 Table 2 The SAP family of SH-2 domain containing adaptors…………….29 Table 3 List of primers, utilized in qRT-PCR experiments………………..214 xi LIST OF FIGURES Figure 1 Systemic administration of EAT-2 expressing adenovirus vector induces cytokine and chemokines responses………….…..............57 Figure 2 Transduction efficiency of innate immune cells by Adenovirus vectors expressing transgenes………….………………………….59 Figure 3 Ad-EAT2-mediated activation of innate and adaptive immune cells in vivo………….………………………...…………………..61 Figure 4 Ad-EAT2-mediated activation of innate and adaptive immune cells in vivo………………………….…………………………….63 Figure 5 IFNγ production from NK cells 6 and 48 hours after Ads injection…………………………………………………………...65 Figure 6 Ad-EAT2-mediated activation of innate and adaptive immune cells in vivo………………………………………………67 Figure 7 HIV-Gag specific cellular immune responses elicited by Ad-HIV/Gag and Ad-EAT2 co-immunization……………………70 Figure 8 HIV-Gag specific cellular immune responses elicited by Ad-HIV/Gag and Ad-EAT2 co-immunization……………………72 Figure 9 Analysis of the breadth of Gag-responses…………........................74 Figure 10 Analysis of T cell epitope responses of Balb/c and C57Bl/6 mice to HIV-Gag in Ad-HIV/Gag and Ad-EAT2 co-injected mice…………………………………………………...76 Figure 11 Cellular immune responses after CD8+ T cells depletion in Ad-HIV/Gag and Ad-EAT2 co-immunized mice……………..…..78 Figure 12 Ad-HIV/Gag and Ad-EAT2 co-immunization increases the frequency of HIV-Gag specific CD8+ T cells………................81 Figure 13 Increased cytolytic activity of the Gag-specific T-cell in vivo in Ad-HIV/Gag and Ad-EAT2 co-immunized mice……....84 Figure 14 EAT2 overexpression augments CD80 and CD86 expression by bone marrow derived macrophages…………..........86 xii Figure 15 Increased expressions of CD40, CD80, CD86, and MHC-II in Ad-EAT2 infected RAW264.7 cells………….................................88 Figure 16 CS protein sequence……………………….....................................99 Figure 17 Ad-CSP construction………….......................................................104 Figure 18 Ad-CSP Stimulates CS protein specific T and B cell responses….107 Figure 19 TLR agonist rEA induced innate cytokines 6 hours post injection………………………………………………………109 Figure 20 Immuno-modulating proteins conversely affect IFNγ secreting splenocytes………….......................................................................111 Figure 21 CS protein expression does not interfere with antigen specific immune responses against other transgenes at low doses………....113 Figure 22 Ad-GFP/rEA combined with 5x107 vp/mouse of Ad-CSP begins to display a diminished CS protein specific CMI response after a dose of 5×106 vp/mouse…………………............115 Figure 23 Co-expression of CS protein and EAT-2 stimulates more potent CS protein specific CMI responses…………………...........118 Figure 24 Expression of GFP does not interfere with CS protein specific CMI responses……………………………......................................120 Figure 25 Co-expression of CS protein and EAT-2 increases the breadth of response against CS protein………………...................123 Figure 26 Improved degranulation of CD8+ T cells in mice co-vaccinated with Ad-CSP and Ad-EAT2………………………125 Figure 27 Co-expression of CS protein and EAT-2 increases cytolytic activity of CS protein specific T cells……......................127 Figure 28 Induction of CS protein specific antibody responses by Ad-CSP vaccines augmented by rEA or EAT-2………………129 Figure 29 Sub-isotype analysis of IgG antibody from plasma of mice co-vaccinated with Ad-CSP and Ad-EAT2…………....................131 Figure 30 CD3+ CD8- IFNγ+ cells respond similarly to both vaccine regimens………………………………………………....133 xiii Figure 31 TRIF acts as a negative regulator of rEA-induced MyD88-dependent activation of dendritic cells in vivo…………..146 Figure 32 TRIF acts as a negative regulator of rEA-induced MyD88-dependent activation of macrophages in vivo……………148 Figure 33 TRIF acts as a negative regulator of rEA-induced MyD88-dependent activation of dendritic cells in vivo (MFI) ……………………………………………………...150 Figure 34 TRIF acts as a negative regulator of rEA-induced MyD88dependent activation of NK, NKT, T, and B cells in vivo………...153 Figure 35 TRIF negatively regulates rEA-mediated MyD88 dependent activation of pro-inflammatory cytokines and chemokines in vivo………..................................................................................156 Figure 36 TRIF negatively regulates rEA-mediated MyD88 dependent activation of pro-inflammatory cytokines and chemokines in dendritic cells……………………………………...159 Figure 37 TRIF negatively regulates cytokine production by DCs, triggered by several common TLR agonists………….....................162 Figure 38 rEA-triggered Erk1/2 phosphorylation is MyD88 dependent. C57BL/6 WT or MyD88-KO mice were injected with 100 ng of rEA……………………………………….164 Figure 39 TRIF acts as a negative regulator of rEA-induced signaling and downstream responses in DCs: model of action……………..170 Figure 40 EAT-2 functions as a negative regulator of Adenovirus mediated induction of CRACC receptor expression on macrophages in vitro……………………………………………………179 Figure 41 EAT-2 over-expression induces similar transcript levels of innate immune responses genes as compared to adenovirus control………….......................................................181 Figure 42 EAT-2 transcript levels and virus protein quantification by BCA……………………………………………………………183 Figure 43 EAT-2 over-expression reduces protein level of CRACC receptor on DCs and macrophages in vitro………….....................186 Figure 44 EAT-2 over-expression negatively regulates CRACC xiv expression in dendritic cells in vivo……………...……….............188 Figure 45 Mutant form of EAT-2 adaptor does not prevent CRACC upregulation by Adenoviruses…………………………..191 Figure 46 EAT-2 requires functional ERK and PLCγ pathways to downregulate CRACC receptor on APCs…………...................194 Figure 47 EAT-2 over-expression induces ERK phosphorylation………….196 Figure 48 EAT-2 is a critical cytokine and chemokines regulator in macrophages……………………………………………...........199 Figure 49 Model of EAT-2 molecular mechanism in APCs………………...205 xv LIST OF ABBREVATIONS Ad Adenovirus ADAR Adenosine deaminase-RNA-specific (IFN-inducible) AIDS Acquired immunodeficiency syndrome ALL Acute lymphocytic leukemia ALT Alanine aminotransferase ANOVA Analysis of variance AP-1 Activator protein-1 APC Antigen presenting cell ASC Apoptosis- associated Speck-like Protein Containing a Caspase Recruitment Domain AsGM1 Anti-asialo GM1 ATCC American type culture collection BCR B cell receptor BMDMs Bone marrow derived macrophages Bcl-2 B-cell lymphoma 2 CAR Coxsackie and adenovirus receptor C3 Complement component 3 CEA Carcinoembryonic antigen CFSE Carboxyfluorescein succinimidyl ester CMI Cell mediated immunity CMV Cytomegalovirus xvi CR Complement receptor CRACC CD2-like receptor activating cytotoxic cells CRAD Conditionally replicative adenoviruses CSP Circumsporozoite protein CTL Cytotoxic T lymphocyte CTLA-4 Cytotoxic T-Lymphocyte Antigen 4 CXCL-9 Chemokine, induced by IFN DAF Decay accelerating factor DAI DNA-dependent activator of interferon regulatory factors DAMP Danger associated molecular pattern DCs Dendritic cells DNA Deoxyribonucleic acid dpi Days post injection DR5 Death receptor EAT-2 Ewing's sarcoma-associated transcript-2 EC Endothelial cells Env gp120 Envelope glycoprotein 120 EDTA Ethylene-diamine-tetra-acetic acid EGTA Ethylene-glycol-tetra-acetic acid ELISA Enzyme-linked immunosorbent assay ELISPOT Enzyme-linked immunosorbent spot assay EM Electron microscopy Erk1/2 Extracellular Signal-Regulated Kinases 1 and 2 xvii ERT EAT-2-related transducer FACS Fluorescence-activated cell sorting FBS Fetal bovine serum Foxp3 forkhead box P3 GAPDH Glyceraldehyde 3-phosphate dehydrogenase GFP Green fluorescent protein G-CSF Granulocyte colony-stimulating factor GM-CSF Granulocyte-macrophage colony-stimulating factor GMP Good manufacturing practice FcγRIIB Fc gamma receptor IIb FDA Food and drug administration HA Hemagglutinin HBV Hepatitis B virus HBsAg Hepatitis B viral surface protein HDAd Helper-dependent adenovirus HEK293 Human embryonic kidney 293 HIV Human immunodeficiency virus HLA Human leukocyte antigen HMGB-1 High-mobility group box 1 protein hpi Hours post injection HSV-1 Herpes simplex virus-1 HVR Hypervariable region of Ad hexon protein ICAM-1 Inter-Cellular adhesion molecule 1 xviii ICS Intracellular staining IFN, IFN Interferons  and  (type I IFNs) Ig Immunoglobulin IL- Interleukins (pro-inflammatory cytokines) IM Intramuscular IP Intraperitoneal IP-10 IFN-γ inducible protein 10 IPS1 IFN-b promoter stimulator-1 IRF3, IRF7, Interferon Regulatory Factors 3 and 7 ISG Interferon stimulatory gene ITAMs Immunoreceptor tyrosine-based activation motifs ITSMs Immunoreceptor tyrosine based switch motifs IV Intravenous JAK-1, JAK-3 Janus kinases 1 and 3 KC (CXCL-1) Keratinocyte derived chemokine, murine analog of human IL-8 KO Knockout LPS Lipopolysaccharides Ly-9, Ly-108 Lymphocyte antigen 9, 108 MAL MyD88-adaptor-like MAPK Mitogen-activated protein kinases MadCAM-1 Mucosal addressin cellular adhesion molecule-1 MHC Major histocompatibility complex ml Milliliter xix MPEC Memory precursor effector cells MPER Membrane proximal external region mM Milli-molar mg Milligram l Micro-liter M Micro-molar g Microgram MCP-1 (CCL-2) Monocyte chemotactic protein 1 MCMV Murine cytomegalovirus MIP-1 (CCL-4) Macrophage inflammatory protein 1 beta MyD88 Myeloid differentiation factor 88 (TLR adaptor) mTOR Mammalian target of rapamycin MFI Mean fluorescent intensity MOI Multiplicity of infection MPL Monophosphoryl lipid A MZ Marginal zone Nab Neutralizing antibody NALP3 NACHT, LRR and PYD domains-containing protein 3 Nef Nuclear factor NFκB Nuclear factor kappa B NHP Non-human primates NK Natural killer NKT Natural killer T cell xx NLRs Nucleotide-binding oligomerization domain/leucine-rich repeat receptors NOD-1, NOD-2 Nucleotide-binding oligomerization domains 1 and 2 NTB-A Natural killer, T and B cell antigen OD Optical density OTC Ornithine transcarbamylase PAMP Pathogen-associated molecular pattern PBMC Peripheral blood mononuclear cells PBS Phosphate buffer saline PCR Polymerase chain reaction PEG Polyethylene glycol PI3K Phosphoinositide 3-kinase PLCγ Phospholipase C gamma PLGA Polyactic glycolic acid PRR Pattern recognition receptor PSF Penicillin, streptomycin, fungizone qRT-PCR Quantitative reverse transcriptase Polymerase chain reaction RANTES Normal T-cell expressed, and secreted RCA Replication competent adenovirus rEA Recombinant Eimeria tenella derived antigen RES Reticulo-endothelial system RIG-1 Retinoid-inducible gene 1 RLR RIG-1-like receptors xxi RNA Ribonucleic acid RORδt Retinoic acid receptor related orphan receptor gamma t SAP SLAM-associated adaptors SARM Sterile α-and armadillo-motif containing protein SD Standard deviation SDS-PAGE Sodium dodecyl sulfate polyacrylamide gel Electrophoresis SEM Standard error of the mean SFCs Spot forming cells SH-2 Src-homology 2 SIV Simian immunodeficiency virus SLAM Signaling Lymphocytic Activation Molecules SLEC Short-lived effector cells SP-A Surfactant protein-A TBK-1 TANK-binding kinase 1 TCID Tissue culture infectious dose TCR T cell receptor TEC Thymic epithelial cells TFH Follicular T helper TGF-β Transforming growth factor beta Th T helper TIR Toll/Interleukin-1 receptor TLR Toll-Like Receptor xxii TNF Tumor necrosis factor alpha TRAIL TNF-related apoptosis-inducing ligand TRAM TRIF-related adaptor molecule TRIM Tripartite motif TRIF Toll/Interleukin-1 receptor (TIR)-domain-containing adaptorinducing interferon-β UV Ultraviolet VCAM-1 Vascular cell adhesion molecule 1 ViViD Violet viability dye VP Viral particle WT Wild-type xxiii Chapter I Introduction 1 1.1 The innate immune system: The innate immune system is conserved across species and represents the first line of general defense against pathogenic infections (1). Engagement and activation of specific components of the innate immune system plays a critical role in the enhancement of T and B cell responses of the adaptive immune system (2). Microbial detection by the innate immune system relies primarily on receptors that recognize a wide range of specific molecular structures present in many microbes known as “pathogen associated molecular patterns” (PAMPs). These PAMPs are detected by the host’s deployment of a wide array of extracellular (secreted), cell surface, or cytosolic molecules, proteins, and receptors generally known as “pattern recognition receptors” (PRRs) (3-5). Unlike the antigen receptors of B and T cells that are somatically generated and clonally distributed, the PRRs are encoded in the germline and not subject to somatic variations. PRRs lack the specificity of the T and B cell antigen receptors. PRRs can also recognize endogenous signals released during host cell stress or death, commonly known as “damageassociated molecular patterns” (DAMPs) (6). Secreted PRRs bind to microbial cell surfaces, activate the complement system, and facilitate opsonization of pathogens for phagocytosis by macrophages and neutrophils (7). The transmembrane PRRs include the toll-like receptors (TLRs) family and the C-type lectins (8). The cytosolic PRRs include the nucleotide-binding oligomerization domain/leucine-rich repeat receptors (NLRs) and the retinoic acid-inducible gene (RIG)-1-like receptors (RLRs) (9). The innate immune system is composed of a network of different cell types expressing or reacting to PRR activation, including: monocytes/macrophages, dendritic cells (DCs), natural killer (NK) cells, NKT cells, neutrophils, gamma delta (γδ) T cells, and mast cells. Each cell of the innate immune system expresses various types of PRRs (9). In addition, the cells of the 2 adaptive immune system, both T and B cells, express multiple innate recognition receptors, implying that pathogen recognition is sophisticated and involves orchestration between innate and adaptive immune cells (10). Innate immune responses are non-specific and more rapid; they occur within minutes to hours following infection, whereas the adaptive immune responses usually take several days to weeks. However, compared to adaptive immune responses that last for a long period of time, the innate immune responses rapidly wane as a result of multiple negative feed-back mechanisms in order to limit the tissue damage that can result from these potent responses (8). The most widely studied and the best characterized family of PRRs are TLRs (4). The transmembrane-located Toll receptor was first identified in the early 1980s in Drosophila. The Drosophila Toll receptor was found to be required for responses to fungal and Gram-positive bacterial infections (11). TLRs were discovered in mammals in mid-1990s. Their discovery gave rise to a tremendous amount of studies, which shed light on TLRs signaling mechanisms associated with the innate immune response. Thirteen mammalian TLRs (10 of which are found in humans) have been identified to date. TLRs are type I transmembrane proteins that variously contain: 1) an extracellular domain containing leucine-rich repeats that mediate the recognition of PAMPs; 2) a transmembrane domain; and 3) an intracellular Toll–interleukin 1 (IL-1) receptor (TIR) domain required for downstream signal transduction (12). The expression of TLRs is cell-type specific, and present in many cell types inclusive of non-hematopoietic epithelial and endothelial cells (EC), macrophages, dendritic cells (DCs), natural killer (NK) cells, and neutrophils (2). TLRs are either expressed on the plasma membrane or in the endosomal/ lysosomal compartments (13). Plasma membrane TLRs recognize conserved cell surface PAMPs, such as 3 Lipopolysaccharide (LPS) of Gram-negative bacteria (TLR4), lipoteichoic acid of Gram-positive bacteria and bacterial lipoproteins (TLR1/ TLR2 and TLR2/ TLR6), and flagelline (TLR5) (13). Endosomal TLRs mainly detect microbial nucleic acids, such as double stranded RNA (dsRNA) (TLR3), single stranded RNA (ssRNA) (TLR7), and dsDNA (TLR9) (13). Five TLRs signaling adaptors have been identified including the Myeloid differentiation primary response gene (88) MyD88, MyD88-adaptor-like (MAL/TIRAP), TIR-domain-containing adaptor protein inducing interferon (IFN)-β (TRIF) (also known as TICAM1), TRIF-related adaptor molecule (TRAM) (also known as TICAM2), and sterile α-and armadillo-motif containing protein (SARM) (14). MyD88 is used by all TLRs except TLR3, MAL is used by TLR2 and TLR4, TRIF is used by TLR3 and TLR4, and TRAM is used only by TLR4 (4). Once activated by a specific ligand, the various TLR interactions with the TLR adaptor proteins trigger a series of intracellular signaling cascades that result in down-stream activation of transcription factors such as nuclear factor (NF)-κB and activated protein-1 (AP-1), leading to transcription of immune response genes and production of pro-inflammatory cytokines and chemokines. In addition, activation of TLRs 3, 4,7,8, and 9 can also result in activation of the interferon regulatory factors (IRFs) 3 and/or 7 signaling pathways, leading to production of type I interferon (IFNs) responses that limit the replication of invading pathogens, as well as the promotion and shaping of pathogen-specific Band T-cell adaptive immune responses (7, 15). 1.2 Adaptive immunity and immunological memory: Unlike the innate immune system that provides critical mechanisms for the rapid sensing and elimination of a wide range of invading pathogens, the adaptive immune system is a system capable of specific recognition, of both self and nonself-antigens, and generation of 4 immunological memory (16). The typical functions of the adaptive immune system are primarily carried out by two cell type: the effector cells, the T lymphocytes, and antibody-producing cells, the B lymphocytes. T lymphocytes mature in the thymus from common lymphoid progenitors derived from the bone marrow or fetal liver (17). In contrast, B lymphocytes mature primarily in the bone marrow (18). Immature T and B lymphocytes go through a positive and a negative selection process. The positive selection process evaluates the ability of antigen receptors of immature lymphocytes to bind to peptide sequences in general, while negative selection processes acts to identify and eliminate cells that are reactive to self-peptides/antigens. Negative selection for immature B lymphocytes occurs in the bone marrow and spleen. Binding to self-antigen in bone marrow leads to deletion, anergy, or receptor editing (18). B cells that survive negative selection in the bone marrow migrate to the spleen, a place where any selfantigen reactive B cells become anergic and destroyed (19). Progenitor T lymphocyte selection on the other hand, occurs in the thymus where thymic epithelial cells (TECs) play a critical role in this process. Positive selection for T cell receptors (TCRs) occurs in the thymus cortex. Negative selection to remove self-reactive immune responsive T cells occurs in the medulla (20). These two selection processes leads to the formation of naïve lymphocytes, with the capability to recognize peptide sequences from foreign, but not self, antigens. In this context, it is important to note here that, negative selection is an incomplete process as it does not remove all selfreactive lymphocytes (21). Thus, a mechanism called peripheral tolerance exists to prevent inappropriate responses by self-reactive lymphocytes that escaped negative selection. One mechanism of peripheral tolerance is induction of anergy in T cells that recognize self-antigens in the absence of co-stimulatory signals (22). Another mechanism of peripheral tolerance is + regulated by a subset of CD4 T-helper cells, called regulatory T cells (Treg), which suppress 5 inappropriate adaptive immune responses to self-antigens via production of immune suppressive cytokines, such as IL-10 and TGF-β (23). Naïve T and B lymphocytes are highly mobile. Following their development in the primary lymphoid organs, they migrate to secondary lymphoid organs, including lymph nodes and the spleen, and then to several sites in the body during infections. Lymphocyte trafficking is facilitated by an array of several adhesion molecules. For example, α4β7 integrins (which binds to mucosal addressin cellular adhesion molecule-1 (MadCAM-1) on gut epithelial cells)expressing lymphocytes, preferentially traffic to the gastrointestinal tract, whereas CLA-1CCR4-bearing lymphocytes home to the skin (24). 1.2.1 T cell activation and cellular immunity: Mature T cells are activated via interaction of their TCR complex with antigenic peptides + complexed with MHC molecules on the surface of APCs. CD8 T cells can interact with peptides (9-11 amino acids in length) presented on MHC class I molecules. These MHC class Irestricted peptides are generally derived from cytosolic proteins (self-proteins or proteins from pathogens that replicate within the cells, such as viruses and intracellular bacteria). In contrast, + CD4 T cells interact with peptides derived from extracellular antigens (approximately 18-20 amino acids in length) presented on the MHC class II molecules. Unlike MHC class I molecules that are constitutively expressed in all nucleated cells, MHC class II molecules are expressed only on APCs in response to inflammatory stimuli, such as the ligands of TLRs (8). The TCR is composed of an α and β protein heterodimer which is non-covalently linked to the signaltransducing CD3 complex. Signaling through the TCR is initiated by the activation of Src protein tyrosine kinases leading to the phosphorylation of CD3 immunoreceptor tyrosine-based 6 activation motifs (ITAMs) followed by recruitment of ZAP-70 and activation of associated + adapter proteins (25). During infections, naïve CD8 T cells are primed by mature APCs in secondary lymphoid organs, leading to their activation and clonal expansion. T cell activation involves dramatic changes in T cell metabolism, such as enhanced uptake of glucose, amino acids, and iron (26). As a result, activated pathogen-specific T cells go through multiple rounds of replication to generate enormous numbers of effector memory T cell population, a population that represent the first line of defense of the adaptive immune response (27). Several receptors and inflammatory mediators have been implicated as necessary to prime naïve T cells for effector functions. For example, signals produced from the TCR-MHC interaction, costimulatory molecules (derived from interactions between other cell surface molecules (such as CD28, SLAM, OX-40, and CD27), and inflammatory cytokines receptors (such as IL-12 and type I IFN), activate several signaling pathways (such as, the PI3K-PDK1Akt-mTOR signaling pathway) and result in T cell proliferation and expansion (28). This process is sometimes as referred to as the three signal hypothesis. Type I IFNs (mainly IFNα) has been + shown to potently enhance CD8 T cell expansion and antigen specific cytotoxicity (29). Further, IL-12, through induction of T-bet via an mTOR-dependent mechanism, has also been + shown to play a critical role in terminal differentiation of CD8 effector memory T cells (30). In addition, besides its role as a T cell growth factor, IL-2 has been shown to also function as a memory T cell differentiation factor by promoting the differentiation of effector memory cells + (31). For example, culturing naïve CD8 T cells in a high concentration of IL-2 facilitate induction of superior effector functions, as compared to cells cultured in low concentrations of 7 IL-2 (32). In addition, the TLRs, IL-1, the IL-18 signaling adaptor protein, and MyD88 have also been recently shown to play a role in effector T cell expansion and survival (33). + Once primed, effector memory CD8 T cells are capable of migrating to inflamed tissue via expression of inflammatory cytokine receptors, such as CXCR3, that allow them to enter and + target to peripheral tissues (34). At the peripheral site of infection, effector memory CD8 T cells kill the antigen expressing infected cells (via Granzyme B and Fas-mediated apoptotic + pathways) and release several cytokines, such as IFNγ, TNFα, and IL-2. Effector memory CD8 T cells have also been shown to have immunoregulatory function. For example, it has been + shown recently that a subset of effector memory CD8 T cells provide immunoregulatory signals via the immunosuppressive cytokine, IL-10, to prevent excessive tissue injury at the site of infection (35). + During the expansion phase, antigen-specific CD8 memory T cells go through many phenotypic and functional changes, including, the re-expression of the IL-7 receptor α, CD127. + Effector memory CD8 T cells are characterized by increased expression of the CD127 (36). Pathogen-specific memory T cells are induced during initial engagement with foreign antigens and are divided into two populations: the short-lived effector cells (SLEC) (characterized by low low expression of ,CD127 ) that mostly die off (90-95%) when the infection is cleared, and the memory precursor effector cells (MPEC) that survive the contraction phase and contribute to long term immunological memory populations (characterized by increased expression of high CD127 ) (37). These CD127 expressing effector memory T cells (TEM) further differentiate 8 into self-renewing central memory T cells (TCM), called “memory stem-cells”, that can persist for extended period of time in the absence of antigenic stimulation (38). Several signaling pathways and cellular mediators have been described to play important roles during the TEM to TCM transition. For example, the Wnt-β-catenin signaling pathway has + + been shown to arrest effector CD8 T-cell differentiation and to promote development of CD8 memory stem cells (39). More recently, the specific inhibitor of mTOR, rapamycin, which has been used extensively as an immunosuppressive drug during tissue transplantations, has been + shown to enhance memory CD8 T cell differentiation during vaccination, confirming a major + regulatory role for mTOR in driving central memory CD8 T cell differentiation (40). Central memory T cells are characterized by increased expression of the anti-apoptotic marker Bcl-2, re-expression of the lymph node homing receptors CD62L and CCR7, and higher expression levels of CD127 (38). In addition, central memory T cells are also characterized by enhanced recall responses to previously encountered antigens and higher protection capacity compared to early-stage memory T cells (38). The survival of memory T cells is dependent on the survival cytokines, IL-7 and IL-15, which maintain the memory cells in a state of slow but continuous proliferation (41). + CD4 T cells are the other group of T lymphocytes. They constitute the largest portion of the T cell population in the body. Most of these cells serve a helper function and are thus designated as T helper (Th) cells. T-helper cells differentiate from naive to effector T cells, a process regulated by interactions with antigen presenting DCs. Following their activation, CD4 + T cells produce a wide range of cytokines that play critical roles in mediating adaptive immunity 9 + to a variety of pathogens. Once activated, CD4 T cells facilitate antibody production by B + lymphocytes (18), enhance and maintain responses by CD8 T cells, regulate macrophage function, enhance the potency of DCs, and regulate immune responses to control autoimmunity and to adjust the magnitude and persistence of immune responses (42). + CD4 T cells are also classified into several effector cell subsets. Based on their + cytokine expression patterns or profile, CD4 T cells (both in mice and human) were initially divided into two major subsets, designated Th1 and Th2 (43). The Th1 cell subset was characterized by their ability to produce their signature cytokine, IFNγ. In contrast, Th2 cells + variously produce IL-4, IL-5, and IL-13. Differentiation of naïve CD4 T cells into effector Th1 type cells is influenced by IL-12 (produced by activated DCs) and IFNγ (produced by activated NK and NKT cells) and is regulated by the T-box expressed in T-cells (T-bet) transcription factor (42). In contrast, Th2 differentiation of these T cells is driven by IL-4 (which is produced by activated T cells and other cells) and the transcription factor GATA-3 (42). Both Th1 and Th2 cells function to eliminate different pathogens in the body. Cytokines produced from Th1 cells enhance cell-mediated immunity of NK cells, macrophages, and cytotoxic T cell to kill and eliminate intracellular pathogens and virally stressed cells (44). Th2 cytokine profile has been shown to enhance humoral immunity, thus helping in eliminating extracellular pathogens and parasites (44). In addition, Th1 and Th2 cells have also been shown to differ in their homing capabilities as a result of differences in the expression pattern of several chemokine receptors. For example, Th1 cells express CCR5 and CXCR3, while Th2 cells express CCR4 and CCR3 receptors (44). 10 In the past few years, several additional subsets of T-helper cells have been described. A subset designated as Th17 has been identified (45). Th17 cells produce IL-17 as their signature cytokine as well as IL-22 and GM-CSF (44). Th17 cells are induced by IL-6 and TGF-β, express the transcription factor RORγt (retinoic acid receptor related orphan receptor γt), have distinct homing capabilities (based on CCR6 expression), and are required for the elimination of fungi and extracellular bacteria (46). The existence of Th2 cells subset that produce high amounts of the cytokine IL-9 in response to IL-4 and TGF- β, are designated as Th9 cells (47). In addition, a subset of T-helper cells, designated Th22 cells, produce IL-22 and express the transcription factor aryl hydrocarbon receptor (48). Th22 cells have been shown to play an important role in the skin due to the expression of skin homing receptors, CCR10 and CLA, and the production of IL-22, a stimulator of antimicrobial peptides by keratinocytes (48). Furthermore, a subset of Th cells called follicular T helper (TFH) cells, that resides in the lymph node and spleen has also + been described. TFH cells are central memory CD4 T cells that express the chemokine receptor CXCR5, which mediates their recruitment to follicles. TFH cells can produce both IL-4 and IFNγ. TFH cells also enhance B cell activation (via T-cell-dependent antigen), which leads to germinal center formation. This facilitates B cell proliferation, immunoglobulin isotype class switching, and affinity maturation of antigen-specific B cells. These processes eventuate in the generation of memory B cells and long-lived plasma cells that produce high affinity somatically mutated antibodies of switched isotypes (49). Another subset of CD4 T-helper cells, designated as Treg cells, selectively produce IL10,as well express the IL-2 receptor α chain (CD25) and the transcription factor forkhead box protein 3 (foxp3) has also been described. Treg cells are divided into two main subsets: naturally 11 + occurring Foxp3 regulatory T cells, which develop in the thymus, and inducible regulatory T + cells, which develop in the periphery from conventional CD4 T cells as a result of specific stimuli, such as regulatory cytokines or immunosuppressive drugs (50). Inducible Treg cells are further divided into three types including: T regulatory 1 (Tr1) cells, which secrete IL-10, TGF-β + producing Treg cells, and inducible Foxp3 regulatory T cells (51). Treg cells suppress T cells responses by either direct or indirect mechanisms. Cell-cell contact and/ or production of the immunosuppressive cytokines, IL-10 and TGF-β, can directly inhibit effector T cells responses, whereas Treg cells can modulate the function of DCs and thereby indirectly inhibit effector T cells responses (52). Several studies have indicated that production of IL-10 and TGF-β by Treg cells suppresses host immune responses and induces self-tolerance, thus limiting the magnitude of effector T cell responses and helping to minimize collateral tissue damage caused by pathogen instigated immune responses (51). For example, deletion or blockade of IL-10 leads to enhanced clearance of Leishmania major parasites in mice (53). Also, reduced pulmonary inflammation and lung injury in a mouse model of Pneumocystis pneumonia infection has been directly linked to the availability of Treg cells (54). Furthermore, it has been shown that depletion of Treg cells prior to vaccination, enhanced anti-tumor immunity and increased the antigen-specific T cell responses to vaccine antigens (55), implicating a critical role for Treg cells in vaccination regimens. 12 1.2.2 B cell activation and humoral immunity: B cell development is regulated by diverse signaling pathways and transcription factors (18). B lymphocyte development and function are regulated by signals transduced through the Bcell antigen receptor (BCR). Engagement of the BCR results in activation of several protein tyrosine kinase (PTK) signaling pathways including the Src-family PTKs (Lyn, Fyn, and Blk), Syk, and the Tec-family member Btk (56). B cell development and function are also regulated by signals transduced by other B-cell-specific cell surface molecules such as CD19, CD20, CD21, CD22, CD23, CD24, CD40, Igα (CD79a), and Igβ (CD79b) (57). Each of these B cell-specific cell surface molecules has distinct functions during B cell development and activation. For example, CD19, which is expressed by all B cell lineages, functions to regulate intracellular signal transduction by amplifying the Src-family kinase activity (57). CD19 functions as membrane adaptor protein, which recruits the signaling molecules Vav, PI3K, and Lyn that activate the PLCγ and MAPK signaling pathways (58). Several reports have shown that CD19CD21 complex is crucial for B cell function. For example, mice lacking either CD19 or CD21 have defect in antibody secretion, germinal center formation, and affinity maturation (57). CD20, + which is a mature B-cell marker, functions as a membrane-embedded Ca2 channel. It is important to note here that, the CD20 monoclonal antibody (ritixumab) is the first FDA approved monoclonal antibody for clinical use in cancer immunotherapy (follicular lymphoma) (18). Another component of the multi-protein cell surface BCR complex is the transmembrane immunoglobulin-αβ heterodimer. Igαβ facilitates the recruitment of Src tyrosine kinases to their cytoplasmic ITAMs domains, and thus is essential for initiating BCR signaling and B cells activation (56). 13 Several transcription factors that regulate early stages of B cell development have been identified. In particular Pax5 transcription factor has been shown to play a critical role for B cell lineage commitment and differentiation (18). For example, Pax5-defecinet mice have been shown to have an arrest in B-cell development (59). In addition, a significant number of Pax5 regulated genes (approximately 170 genes) have been shown to play an important role in B-cell signaling, adhesion, and migration (60). Furthermore, Pax5 has also been implicated in human B cell malignancies, as several cases of acute lymphoblastic leukemias (ALL) and non-Hodgkin lymphomas have been shown to harbor somatic PAX5 mutation (chromosomal translocations) (61). The B cell response is initiated at the boundary between T and B-cell areas in the spleen and lymph nodes. Specific antigen recognition by the BCR is the first step in the initiation of B cell signaling and activation. Interactions between B cells, that have captured and processed antigen, and activated T cells, which have been primed by follicular DCs, lead to the expansion of antigen specific B cells and to their differentiation into short-lived plasma cells, which produce low-affinity antibody of IgM or IgD isotype without somatic mutation (62). The formation of GC reaction follows this extra-follicular response. Further signals from follicular helper T cells at the GC result in B-cell proliferation, isotype switch, and affinity maturation of antigen-specific B cells, leading to the generation of memory B cells and long-lived plasma cells that produce high affinity somatically mutated antibodies of switched isotypes (IgG, IgA, or IgE) (49). Germinal center plasma cells then migrate to bone marrow and continually secrete antibodies, thus maintaining constant levels of protective antibodies. Plasma cell migration to bone marrow is regulated by the bone marrow stromal cells, which provide the attracting chemokine CXCL12 and the survival cytokines, such as IL-6 (63). 14 Several B cell subsets with distinct functions have been identified. B-1 and marginal zone + (MZ) B cell subsets have been described in murine models. Murine B-1 cells are a CD5 B cell subpopulation which differ from conventional B cells (B-2) by their phenotype, localization, and + - self-renewing capacity (18). B-1 B cells are further divided into B-1a (CD5 ) and B-1b (CD5 ) cell subsets (64). B-1a subset functions to provide IgM antibodies during innate immune responses against bacterial infection. While, B-1b cells subset provide long-term adaptive antibody responses during bacterial infections (65). MZ B-cells, which are located in the periarteriolar lymphoid sheath of the murine spleen, function as the first line of defense against blood-born encapsulated bacteria (66). In addition to B-1 and MZ B cells, several subpopulations of B cells in peripheral blood have also been characterized according to specific surface marker + - - + + - + + + expression, such as IgM IgD CD27 (immature), IgM IgD CD27 (naïve), IgM IgD CD27 - - + (marginal zone, unswitched memory), IgM IgD CD27 (germinal center, switched memory), high CD38 IgM high (activated), and CD38 high - IgM (plasmablast) (16). More recently, a subset of rare antigen-specific regulatory B cells (B10) with a unique phenotype high (CD1d + CD5 CD19 high ) has been identified in mice spleens (67). B10 cells have been shown to play critical regulatory role in the immune system via IL-10 production. 1.3 Regulation of adaptive immunity by the innate immune system: The cells of the adaptive immune system cannot typically recognize most antigens by themselves; their responses are usually modulated by innate immune cell PRR-induced signals. Therefore, innate and adaptive immunity work together to effectively target the adaptive immune 15 responses toward pathogens and allow the adaptive immune system to distinguish self from nonself. Triggering the activation and maturation of DCs via recognition of PAMPs by PRRs is the most widely studied mechanism that bridges innate and adaptive immunity (68). Recent advances in the field of innate immunity however, have identified critical roles for other innate immune PRRs, as well as other innate immune cells, in orchestrating the function of both innate and adaptive immune responses. Here I will provide background about the role of various innate immune cells in adaptive immune cell regulation, cross-talk between innate and adaptive immune systems, and the impact of various innate immune receptors signaling pathways on adaptive immune cell responses. 1.3.1 Innate immune cell-dependent regulation of the adaptive immune system: The expression and production of cytokines upon recognition of PAMPs or endogenous danger signals by cells of the innate immune system, including monocytes/macrophages, DCs, NK cell, NKT cells, and neutrophils, play a critical role not only in innate immunity but also in immune regulation of the adaptive immune system (2). DCs are the major antigen-presenting cells of the immune system that have an important role in the induction and regulation of immune responses against pathogens (69). Through expression of several PRRs such as TLRs and C-type lectins, DCs detect and initiate innate and adaptive immune responses that lead to pathogen elimination and/or control. In addition, DCs provide stimulatory signals and interacting with several cells of the innate and adaptive immune systems, such as NK-, NKT-, T-, and Bcells (70). Activated cDCs produce several cytokines that are crucial for promoting cytotoxic T lymphocyte (CTL) response (via IL-12) and enhancing NK cell activities and survival (via IL-12, IL-15, and IL-18) (71). In the absence of inflammatory stimuli, DCs are in an immature stage 16 and induce tolerogenic T cell responses (72). However, in an inflammatory microenvironment, such as in the presence of TLR ligands and inflammatory cytokines and chemokines, DCs have an enhanced ability to capture antigens, mature, and migrate to lymph nodes, where antigenspecific adaptive immune responses are induced (72). DCsꞌ maturational processes involve expression of MHC class II, co-stimulatory molecules, such as CD80 and CD86, lymph node homing receptors, such as CCR7 and CD62L, and production of pro-inflammatory cytokines and + + chemokines, such as IL-12, type I IFNs, and TNFα (73). DCs can activate CD4 and CD8 T + + cell responses either by direct antigen-presentation to CD4 or CD8 T cells or by cross+ presentation to CD8 T cells (73, 74). Moreover, it has been proposed that, in addition to their immunostimulatory function, DCs have an immunoregulatory function leading to suppression of T cells responses and control of excessive inflammatory reaction (75). NK cells have also been shown to play critical roles in shaping adaptive immunity. Several reports have demonstrated that, in addition to their function in the innate immune response, NK cells play an important role during the induction of adaptive immune responses. For example, IFNγ production from NK cells have been shown to enhance cDCs maturation and promote the differentiation of DCs that are capable of inducing efficient CTL responses (76). In addition, NK cells were shown to bridge innate and adaptive immune responses by providing signals for augmenting Th-1 immune responses by production of IFNγ in response to inflammatory stimuli (77, 78) and inducing tumor-specific CTLs (79). In addition, it has been shown recently that NK cell-mediated cytotoxicity of antigen-expressing target cells triggers DCs cross-presentation and thus, facilitates induction of robust antigen-specific adaptive immune responses (80). 17 NK cells have also been shown to play critical role for B cell activation and the promotion of isotype class switching. For example, B cell activation by NK cells has been shown to trigger the production of antigen-specific antibodies with IgG2a and IgG1class-switch isotype (81). NK cell depletion, via administration of PK136 anti-NK1.1 antibody, has been shown to affect both the number and the maturation state of DCs in the lymph node (82). Moreover, depletion of NK cells has also been shown to inhibit the generation of anti-tumor T-cell responses (79). Interestingly, NK cells have also been shown to have inhibitory roles during adaptive immune responses. For example, depletion of NK cells after murine cytomegalovirus (MCMV) infection has been shown to result in enhanced proliferation of CD8+ T cells and + + increased production of IFNγ by both CD4 and CD8 T cells (83). Additionally, depletion of NK cells have been shown to enhance the tumor-specific CTL responses to MHC class I positive lymphoma (84), demonstrating that NK cells have both immuno-stimulatory and immunoinhibitory roles during adaptive immune responses. It is important to note here that depletion of NK cells by NK1.1 antibody can deplete other cells that express the NK1.1 molecule, such as NKT cells, thus a role for other cells expressing the NK1.1 cannot be excluded in these types of experiments. Indeed, several reports have demonstrated that enhancing the activation of NKT cells can also positively influence the initial activation of DCs and/or NK cells, thereby increasing DC-dependent anti-tumor and anti-viral adaptive immune responses (85-89). In addition to DCs and NK cells, neutrophils have also been described to play critical role in bridging innate and adaptive immune responses (90). Neutrophils have been shown to produce several cytokines and chemokines (such as, IL-1α, IL-1β, IL-6, IL-10, TGFβ, IL-12, IFNα, IFNγ, G-CSF, and GM-CSF) that are crucial for regulating innate and adaptive immunity (90). For example, neutrophil derived cytokines have been shown to induce DC maturation and to enhance 18 IL-12 and TNFα production from DCs both in vitro and in vivo (91). Neutrophils have also been found to directly stimulate the production of IFNγ by human NK cells, thus influencing DC maturation and Th-1 immune responses (92). Furthermore, human neutrophils have been shown to directly interact with B- and T-cells to influence their functions (93). Human neutrophilsderived cytokines, BAFF and APRIL, have been shown to be crucial for the survival, maturation and differentiation of B lymphocytes (94). In addition, the neutrophil-derived chemokines CCL2, + CCL20, CXCL9, and CXCL10 have been shown to attract CD4 Th-1 and Th-17 cells to sites of inflammation (95). Furthermore, human and mouse neutrophils have been shown to crosspresent exogenous antigens, as immunization of mice with OVA-pulsed neutrophils promoted + the differentiation of naive CD8 T cells into antigen-specific cytotoxic T cells (96). A critical component of the innate immune system, the complement system, not only plays a central role in innate immunity, but it has also been described to play critical roles in adaptive immune cell responses. Complement, in particular C3, has been shown to modulate Band T-cells responses (97). For example, administration of monoclonal or poly-clonal antibodies to the C3 receptor, complement receptor 2 (CR2), on human B cells, resulted in B cell proliferation and differentiation (98). Notably, CR2 is expressed on several innate and adaptive immune cells including mature B cells, follicular DCs, thymocytes, and sub-population of T cells (98), implicating a role for C3 in the function of these immune cells. Opsonisation of several antigens with C3d has been shown to increase their immunogenicity and to lower the threshold of B cell response to the coated antigen by 1000-fold (99). Mice deficient in CR2, CR1/2 KO mice, have abnormal B cell survival and impaired B cell functions including reduced levels of natural antibodies, smaller germinal centers, and irregular humoral immune responses to antigens (100). Similarly, work in our lab and others have also demonstrated a critical role for CR2 in B 19 cells responses. We have demonstrated that CR1/2 KO mice exhibited impaired B cell responses following adenovirus administration (101). The human decay-accelerating factor (DAF), a natural complement system inhibitor, was shown to retain anti-complement activity when displayed from the surface of the Ad capsid in a retro-oriented fashion (102). Studies in our lab have shown that mice injected with the “DAFdisplaying” Ad5 vectors, demonstrated significant reductions in pro-inflammatory cytokine release, reduced endothelial cell activation, minimized activation of pro-inflammatory genes expression, and reduced plasma ALT levels in mice as compared to unmodified Ad5 vectors. Also, these results correlated positively with a significantly decreased activation of dendritic cells, NK cells and T cells (102, 103). Importantly, this modulation of the complement dependent arm of the innate immune response resulted in significantly reduced induction of Ad neutralizing antibody responses, as well as in blunted T cell responses to the transgene (HIV/Gag) expressed by the DAF displaying Ad (103). The role of complement in T cell responses has also been demonstrated. Several reports have demonstrated that DCs express a number of receptors specific for complement proteins (CR3, CR4, C3aR, and C5aR) and ligand binding to these receptors can affect DC maturation and migration. For example, interaction of C5a with C5aR on DCs, has been shown to induce DCs maturation and expression of the lymph node homing chemokine receptor CCR7, implicating an indirect role for C5a-C5aR interaction in T cell activation (104). Similarly, binding of C3a to C3aR on DCs has been shown to result in IL-12 production, which supports Th1 immune responses (105). In addition, C3aR KO mice have been shown to have increased production of the Th2 immune response cytokines IL-4, IL-5, and IL-10 (105), suggesting a role for C3a-C3aR interaction in mediating Th1 immune responses to antigens. Complements have 20 also been shown to have immuno-inhibitory roles. For example, iC3b has been shown to inhibit DCs maturation (inhibit expression of CD40 and CD86) and to inhibit production of the proinflammatory cytokines IL-1β, TNFα, and IL12 by DCs (106). 1.3.2 The impact of innate immune recognition on adaptive immune cell responses: Several PRRs act as signaling receptors to induce production of innate effector molecules upon pathogen encounter, or when host-derived markers of stress and/ or damage, are present. These signaling receptors are divided into several classes including TLRs, RLRs, and NLRs (107). Recognition of PAMPs by TLRs results in activation of NFκB/ AP-1 signaling pathways, leading to production of pro-inflammatory cytokines and chemokines (such as IL-6, IL-12, and TNFα) that coordinate innate immunity and initiate adaptive immune responses to various pathogens (8). In addition, TLRs 3, 4, 7, 8, and 9 can activate the IRF3 and/ or IRF7 signaling pathways in pDCs during viral infection and result in the induction of multiple genes involved in innate and adaptive immunity, including type I IFNs (IFNα and IFNβ) (8). Induction of type I IFNs have been shown to result in activation of NK cells (increased IFNγ production and cytotoxicity), enhancing DC maturation (increased, IL-12 production, expression of MHC-II, and costimulatory molecules), and induction of Th1 immune responses (108). In addition, type I IFNs can directly activate adaptive immune cells. For example, type I IFN receptor signaling in CD8+ T cells was found to be critical for the generation of effector and memory CD8+ T cells in response to viral infection (109). Innate immune signaling has also been shown to suppress the adaptive immune system. For example, TLR2 signaling in DCs has been shown to produce high levels of IL-10 and little IL-12, thus promoting the differentiation of Th2 cells (which activate humoral immunity) or regulatory T cells (which suppress cellular immunity) (110). 21 In addition to TLRs, members of the C-type lectin family of cell surface PRRs, called Dectin-1 and 2, have also been shown to regulate adaptive immunity. Dectin-1 recognizes βglucans from fungal pathogens, such as Candida albicans (111). Dectin-1 stimulation has been shown to induce DCs maturation and the production of Th-17 supporting cytokine, IL-23 (112). In addition, when used as an adjuvant, Dectin-1-activated DCs have been shown to favor the differentiation of T cells into the Th17 phenotype, which is required to clear fungal infection (112). In addition to transmembrane receptors on the cell surface and in endosomal compartments, several studies have shown that intracellular (cytosolic) PRRs, such as RLRs and some NLRs, can also regulate adaptive immune responses (113). Innate immune sensing of peptidoglycan by NOD1 and NOD2 proteins has been shown to contribute significantly to the initiation of the adaptive immune responses. For example, NOD1 stimulation alone has been shown to drive antigen-specific immune responses toward Th2 phenotype (114). In addition, antigen-specific T- and B-cell immune responses were severely diminished in NOD1 KO mice following vaccination (114). In contrast, NOD2 has been shown to mediate the adjuvant effect of muramyl peptide (MDP) and regulate bacterial immunity within the intestine (115). The NLR NALP3 inflammasome has also been shown to play a role in regulating adaptive immune responses. Several adjuvants such as aluminum hydroxide (alum), infections such as influenza, or products of cell death such as extracellular ATP and uric acid have been shown to activate the NALP3 inflammasome (116). Activation of the NALP3 inflammasome in DCs has been shown to activate caspase-3, trigger IL-1β release, and induce anti-tumor adaptive immune responses (117). 22 The RLRs RIG-I and MDA5 have also been shown to play critical role in antiviral immune responses. Recognition of viral RNA by the helicase domain of RIG-I and MDA5 leads to the activation of NFκB and IRF3, thus trigger transcription and production of proinflammatory cytokines and chemokines, type I IFNs, and induction of Th1 adaptive immune responses (118). + A cytosolic DNA sensor has also been shown to enhance cytotoxic CD8 T cell and antibody responses following DNA vaccination in a TANK-binding kinase 1 (TBK-1) and type I IFNdependent manner (119). 1.4 Vaccine Adjuvants: Vaccine adjuvants are represented by different classes of compounds including, microbial products, mineral salts, emulsions, nucleic acids, and liposomes (120). Vaccine adjuvants were traditionally used to enhance the immunogenicity of vaccine antigens. Adjuvants have been found to increase the speed of initial response (jump-start innate immunity), increase the generation of immunological memory, alter the breadth of the response, and provide specific + + types of immune responses, such as Th1 versus Th2 or CD8 versus CD4 T cell responses (120). The cellular and molecular mechanisms of actions of adjuvants are poorly understood (120). Vaccine adjuvants can be classified into two major groups, TLR-dependent and TLRindependent (121). TLR-dependent adjuvants act directly on DCs and results in up-regulation of MHC class II, increased expression of costimulatory molecules, production of pro-inflammatory cytokines and chemokines, and enhanced migration of DCs to T cell area in lymph nodes (122). Examples of TLR-dependents adjuvants that are currently in preclinical or clinical studies include, the synthetic analogs of dsRNA, such as Poly I:C (TLR3) (123), monophosphoryle lipid A (MPL) (TLR4, currently licensed for HBV and papilloma vaccines) (124), bacterial flagelline 23 (TLR5) (125), Imiquimod (TLR7) and Resiquimod (TLR7-TLR8) (126), and CpG-ODN (TLR9) (127). The TLR-independent adjuvants include alum and the squalene-based oil-in water emulsions MF59 and AS03. These latter adjuvants are widely used in human vaccines (128). While the molecular mechanism of action and the target cells of these adjuvants are still unknown, several studies have found that these adjuvants enhance antigen up-take by APCs (129). However, recent studies suggested that, in addition to antigen delivery, these adjuvants might have immunostimulatory functions in vivo. For example, Alum has been found to activate the NALP3 inflammasome to produce mature IL-1β and to trigger necrotic cell death and the release of the endogenous danger signal, uric acid (120). In addition, MF59 has been found to stimulate the release of lymphocyte chemoattractants, such as CCL2, CCL3, CCL4, and CXCL8, from human macrophages, monocytes, and granulocytes in vitro (130). In addition, MF59 has also been shown to enhance monocyte differentiation into DCs in vitro (130). Similar to alum, when administered in vivo, MF59 has been found to trigger a local immunostimulatory environment, an environment that resulted in differentiation of monocytes into inflammatory DCs expressing high levels of MHC class II molecules. 1.5 The immuno-modulatory molecule: recombinant Eimeria Tenella derived Antigen (rEA) The bovine small intestine is resistant to tumor formation, and an activity isolated from that small intestine was isolated and identified as a protein derived from Eimeria tenella. A recombinant form of this Eimeria tenella derived antigen (rEA) has been generated. (131). The EA originates from an endemic pathogenic protozoan of the Eimeria genus Apicomplexa phylum 24 that include Toxoplasma, Plasmodium, Eimeria and cyclospora genera. rEA has been shown to induce IL-12 release from mouse DC in vitro and to trigger potent production of IL-12 and other proinflammatory cytokines and chemokines in vivo (131, 132). rEA has also been shown to trigger potent IFNγ release from NK cells and to enhance NK cytolytic activity toward S-180 tumor cells both in vitro and in vivo, an activity that was completely dependent on IL-12 release from DCs (131). Studies in our lab have also shown that NK and NKT cell activation was significantly induced following systemic rEA administration (133). The enhanced activation of DCs and NK cells as well as the increased production of proinflammatory cytokines and chemokines by rEA positively correlated with enhanced humoral and Th1 cellular immune responses to the co-administered Toxoplasma gondii antigen, resulting in protection against Toxoplasma gondii infection in mice (134). rEA has also been shown to be an efficient immunomodulator, having both anti-viral and anti-tumor properties in mice (131, 135, 136). Moreover, rEA showed no evidence of toxicity in pre-clinical (131) and clinical trials (137). Specifically, in a phase I human clinical trials, rEA was used as a single agent in advanced gynecological cancer patient. The results from the trial showed a reduction in CA125 levels as indication of treatment-associated efficacy and importantly, no severe adverse reactions were reported in human clinical trials despite detection of increased IL-12p70 responses in up to 30% of the treated cancer patients (137). The rEA protein has a relatively high amino acid sequence homology (67%) and shares very similar biological activities in vitro and in vivo with T. gondii-derived profilin-like protein, both of which trigger potent IL-12 responses in DCs. The profilin induced responses were completely dependent upon the adaptor protein MyD88 and at least partially mediated via TLR11 (138). Moreover, it has been shown in vitro that human TLRs (TLR2, TLR3, TLR4, 25 TLR5, TLR7, TLR8 and TLR9) do not transduce rEA signaling (135). Therefore, TLR11 has been suggested as the rEA receptor mediating rEA signaling, but this notion remains to be confirmed. Since no functional human TLR11 homolog has been discovered, these facts leave unidentified the mechanism underlying rEA action in humans, and opens a discussion regarding other PRRs that may be involved in rEA signaling (135). Additionally, it is not known what cell types are primarily responsible for mediating rEA-triggered responses in vivo. Studies in our lab have shown that the magnitude and quality of HIV-Gag-specific T cell responses were significantly improved when rEA expressing Ads and antigen expressing Ads were administered together (139). In addition, inclusion of rEA in the Ad based vaccination regimen improved the + in vivo cytolytic activity of the HIV-Gag-specific CD8 T cells (102). 1.6 Harnessing innate immunity by the SLAM family of receptors adaptor EAT-2 1.6.1 The signaling lymphocytic activation molecules (SLAM) family of receptors The SLAM family of receptors is a group of type I transmembrane receptors that play an important role in immune regulation (140). These receptors belong to the CD2 superfamily of immunoglobulin (Ig) domain-containing molecules, are expressed on the surface of wide variety of immune cells, and are not found in non-immune cells. SLAM family of receptors includes SLAM (CD150; SLAMF1), 2B4 (CD244; SLAMF4), Ly-9 (CD229; SLAMF3), natural killer, Tand B-cell antigen (NTB-A) or Ly108 (in the mouse) (SLAMF6), CD84 (SLAMF5), and CD2like receptor activating cytotoxic cells (CRACC; CD319; and SLAMF7) (Table 1) (141). 26 Table 1: Expression pattern of SLAM family of receptors in hemopoietic cells Table 1 Receptors Physiological ligand SLAM SLAM 2B4 NTBA/L108 CD48 NTBa/Ly108 Ly-9 Ly-9 CD84 CD84 CRACC CRACC Cellular distribution T, B, DCs, Platelets, and Macrophages NK cells, CD8+ T cells, DCs, and Macrophages NK, DCs T, and B cells NK, DC, neutrophils, B, and T cells T, B, NK, DCs, Platelets, and Macrophages T, B, NK, DCs, and Macrophages Associates with SAP, EAT-2, ERT, SHP2, SHIP1, and FYN SAP, EAT-2, ERT, SHP2, SHP2, and LAT SAP, EAT-2, ERT, SHP2, SHP2, and LAT SAP, EAT-2, ERT, SHP2, and AP2 SAP, EAT-2, ERT, SHP2, and SHIP1 EAT-2, SHP1, SHP2, and SHIP1 Structurally, SLAM family of receptors contain an extracellular domain composed of two immunoglobulin (Ig)-like domains, a transmembrane domain, and a cytoplasmic domain that carry one or more copies of a unique intracellular tyrosine-based switch motif (ITSM) (141). The SLAM family of receptor ITSM motif has a high affinity for a family of adaptor molecules called SLAM-associated protein (SAP) family of adaptors. The SLAM receptors have a unique feature compared to most other receptors expressed in immune cells, in that they are self-ligands. One exception is 2B4, which interacts with CD48, a member of the Ig superfamily that is expressed in most hematopoietic cells (142). Thus, SLAM family of receptors can be triggered by homotypic or heterotypic cell-cell interactions through their respective extracellular domains. Several lines of experimental investigation have been utilized to identify the role of these receptors in immune cells including, antibody stimulation approaches, ectopic expression of ligands on target cells, genetic linkage analysis, and analysis of genetically modified mice (140). Recent data suggest that these immuno-modulatory receptors perform multiple functions in 27 + hematopoietic cells, including roles in regulating cellular costimulation, NK- and CD8 T-cellmediated cytotoxicity; macrophage, DCs, T cell and NK cell cytokine production; adhesion between hematopoietic cells; the development of innate T lymphocytes; as well as regulating functions of neutrophils and macrophages (143, 144). The SLAM family of receptors genes are located within a 400 kilobase (kb) fragment on chromosome 1 in both humans and mice (145). The similarities between SLAM family genes in sequence, genomic organization, gene localization, and ITSMs, imply that the SLAM family was generated by sequential duplication of a single ancestral gene. Several reports demonstrated the presence of multiple splice forms and polymorphisms for many of the genes encoding the SLAM family members. For example, polymorphisms of the Ly108-encoding gene, in mice, were found to correlate with susceptibility to the autoimmune disease systemic lupus erythematosus (SLE) (146). Furthermore, polymorphisms in the Ly-9 and 2B4-encoding genes were respectively linked to susceptibility to SLE and rheumatoid arthritis (RA) in humans (147, 148). 1.6.2 Signal transduction of SLAM family of receptors SLAM family of receptor initiated intracellular signaling is mediated primarily by the SAP family of adaptors. The SAP family of adaptors are composed of three members named, SLAM-associated protein (SAP), Ewing's sarcoma-associated transcript-2 (EAT-2), and EAT-2related transducer (ERT) (Table 2) (149). Table 2: The SAP family of SH-2 domain containing adaptors: The SAP family of adaptors are mainly expressed in immune cells, with SAP present in T cells, NK cells, NKT cells, and some B cells. EAT-2 is found in NK cells, DCs, and macrophages, 28 while ERT is expressed only in mouse NK cells. The SAP encoding gene is located in the X chromosome in humans and mice while EAT-2 and ERT are located on human chromosome 1. The ERT encoding gene is a pseudogene, being nonfunctional in humans. Table 2: The SAP family of SH-2 domain containing adaptors: Table 2 Chromosomal location X (human and SAP (SH2D1A) mice) 1 (human and EAT-2 (SH2D1B1) mice) 1 (mice and pseudogene in ERT (SH2D1B2) human) Adaptor Expression pattern T, B, NK, NKT, and platelets NK, DCs, and macrophages Signaling partners FynT, PIX, and Nck (arginine 78) ? PLCγ (Cterminal tyrosine) NK cells ? (C-terminal tyrosine) Human disease XLP none none These intracellular adaptor molecules are composed primarily of a Src-homology 2 (SH2) domain, in addition to a short carboxy-terminal region. Through their SH-2 domain, SAP adaptors associate with the tyrosine-based switch motif TI/VYxxV/I in the cytoplasmic domain of SLAM receptors with high affinity and specificity (141). All SLAM receptors can interact with either of the adaptors, except CRACC which interacts only with EAT-2 (150, 151). The importance of the SAP adaptors and SLAM receptors was indicated in 1998 after the discovery that SAP is mutated and inactivated in 60%-70% of cases of a human immunodeficiency, X-linked lymphoproliferative (XLP) disease (152). XLP patients have a number of distinguishing features characterized by ineffective responses to Epstein-Barr virus (EBV) infections and a higher frequency for development of malignant lymphomas (152). This is correlated with defects in the development and function of several immune cell types, + + including CD4 T-, CD8 T-, NK-, NKT-, and B-cells (140). Similar to SAP-deficient humans, mice lacking one or more members of SAP family adaptors have a similar phenotype (153). SAP 29 + deficient mice have defects in CD4 T cells function, including Th2 cytokine production and defective TFH cell function, which impact upon germinal center formation and antibody + production by B cells (154). In addition, SAP deficient mice have reduced CD8 T- and NKcells cytotoxic capabilities, absence of NKT cells, and reduced IFNγ production by NK cell (155). Mutation of EAT-2 or ERT was not described in XLP disease, however, mice lacking EAT-2 or ERT have been shown to have altered NK cells functionality (156). In addition, work by the Veillette et al group showed that mice lacking all SAP family of adaptors have severe NK cells defects as compared to mice lacking individual SAP family adaptors (157). SAP and EAT-2 adaptors have been shown initially to prevent the binding of inhibitory signaling molecules to the SLAM family of receptors ITSMs (158). These molecules include, the SH-2 domain-containing phosphatases (SHP)-2 and (SHP)-1, Csk, and SH-2 domain-containing 5’ inositol phosphatase (SHIP)-1 (153). This led to the conclusion that SAP adaptors are natural blockers that allow SLAM family of receptors to mediate positive immune cell activation. However, subsequent studies showed that SAP, EAT-2, and ERT harbor specific sequences that allow them to couple SLAM family of receptors to active biochemical signaling molecules (140). For example, the SAP adaptor has been shown to have a specific sequence within the SH-2 domain, arginine 78 (R78) motif which associates directly with the protein tyrosine kinase FynT (159). This association links SLAM family of receptors to protein tyrosine phosphorylation signals (160, 161). In addition, activation of SLAM (CD150) receptor signaling in B and T cells by antibody crosslinking experiments resulted in PI3K activation and enhanced Akt + phosphorylation (162, 163). Furthermore, SLAM-SAP signaling in CD4 T cells prolongs 30 protein kinase C theta (PKCθ) recruitment to the site of contact with antigen presenting cell and resulted in increased NFκB activation (164). EAT-2 and ERT do not contain the arginine (R87) motif that is found in the SAP adaptor, however, similar to SAP, EAT-2 and ERT have been shown to transduce positive signals downstream of SLAM family of receptors in human and mouse NK cells (144, 150). The activation function of EAT-2 and ERT was dependent on phosphorylation of tyrosine residues directly located in their short carboxyl-terminal tails (165). Mouse EAT-2 and ERT contain two tyrosine residues (tyrosine 120 and 127) in their short C-terminal tail (156). In contrast, the human EAT-2 adaptor contains only one tyrosine residue (tyrosine 127) in its C-terminal tail (144). Phosphorylation of EAT-2 and ERT tyrosine residues serves as a docking site for other, yet unidentified, SH-2 domain-containing downstream effector molecules (166). Recent data suggest that phospholipase C (PLC)-γ is recruited to tyrosine 127 in human EAT-2 (167). Furthermore, over-expression and binding studies indicate that EAT-2 directly binds to the catalytic domain of the Src family kinases, Fyn, Hck, Lyn, Lck, and Fgr (168). It is important to note here that, the role of the EAT-2 adaptor in immune cell function has been studied primarily in human and mouse NK cells. Whether EAT-2 or ERT harbor critical function in immune cell types other than NK cells, still needs to be clarified. 1.7. Adenoviruses as vaccine vectors: Replication-deficient adenovirus based vectors (Ads) have been the focus of considerable interest in the last few years for their potential applications in both gene therapy and vaccine developments (169). Adenoviruses are a family of non-enveloped viruses containing an icosahederal protein capsid with a 30- to 40-kb linear double-stranded DNA genome. In general, 31 of the immunologically distinct human Ad serotypes, none are associated with any neoplastic disease, with most causing relatively mild, self-limiting respiratory illnesses in immunocompetent individuals (170). At least 51 serotypes of human Ad have been identified, which are categorized into six subgroups (A-F), primarily based upon different red blood cell agglutinating capabilities of the various subgroups. Ad serotypes 5 (Ad5) and Ad2, both belonging to subclass C, are the most extensively studied and characterized both relative to general Ad biology, as well in regard to utilization as a gene transfer vector. Several generations of recombinant Ad vector has been generated. First generation adenovirus vectors are constructed such that a transgene replaces only the E1 region of genes ([E1-] Ad) or E1 and portion of E3 ([E1-, E3-] Ad); thus, 90% of the wild-type Ad genome is retained in the vector. Ad vectors can be propagated in human cells (HEK 293 cells) engineered to express the E1 proteins in trans (171, 172). Ad vectors with additional deletions in their genome in the E2A, E2B and E4 Ad genes have been generated (accommodated by use of newer generation, trans-complementing packaging cell lines) (173-176). These advanced generation Ad vectors produce fewer Ad derived gene products as compared to first generation Ads, and can minimize the induction of vector-specific adaptive immune responses (174, 177). Ad vectors posses several important advantages, the most important of which is that they can be easily and routinely produced to high titers in a good manufacturing practice (GMP) 13 compliant fashion (up to 1×10 vp/ml). Additionally, Ad vectors allow for efficient transduction of various proliferating and quiescent cell types, they can allow for transfer of large segments of foreign DNA (up to 35 kB in some systems), and most importantly, Ad vectors do not integrate, and therefore are much less likely to cause insertional mutagenesis or germ line transmission 32 associated problems, in contrast to integrating virus based vectors, such as retrovirus and lentivirus based gene transfer systems (178). Ad vectors rapidly activate innate immune responses as well induces potent cellular and humoral adaptive immune responses, against both the vector and transgene product being expressed (179-181). Upon initial introduction into a host, the innate immune response can be initiated following the binding or coating of the Ad vector capsid with several (extra-cellular) factors including: surfactant-A (SP-A), lactoferrin, pre-existing immunoglobulin, and protein members of the complement pathways, both classical (C1q, C4) and alternative (Factor B, Factor D) (182, 183). Administration of Ad vectors results in the immediate production (1-6 hours post injection) of various pro-inflammatory cytokines and chemokines, as well as type I IFNs in mice, non-human and human primates (179, 181, 184-186). Specifically, high dose, intravascular administrations of Ad vectors have been found to induce high levels of the cytokines IL-1α, IL1β, TNFα, IL-6, IL-12, and IFNγ and the chemokines RANTES, MCP-1, KC, MIP-1α, MIP-1β, and IP-10 (133, 181, 184, 187). The origins of these pro-inflammatory mediators in vivo is not fully known but is likely from multiple sources inclusive of Kupffer cells, macrophages, endothelial cells, as well as Ad transduced tissues and organs themselves (184, 188). Furthermore, Ad vectors can either activate of directly transduce various immune cells types in the liver and spleen including: dendritic cells (189), both pDCs (185) and cDCs (190), macrophages (191), and to a lesser extent NK cells (191, 192). The induction of type I IFNs is critical for innate immune defense against Ad vectors in vivo (193); the maturation of DCs (194); and the regulation of the induction of pro-inflammatory cytokines (195, 196). 33 1.7.1 Molecular basis for cellular recognition of Adenovirus vectors Ad5 vectors interact with both the Coxsackie-Adenovirus receptor (CAR) (via the Ad fiber knob domain) and with cellular integrins (via the Ad penton base RGD motifs) to initiate host cell penetration (197, 198). The penetration process itself will also simultaneously trigger cellular pro-inflammatory innate immune responses (199). After internalization and upon endosomal escape, Ad vectors have been shown to activate MAPK and NFκB signaling pathways via both TLR dependent and TLR non- dependent mechanisms (184, 195). We and others have shown that, Ad vectors activate TLR (TLR2, TLR4, and TLR9) signaling and induce various cytokines and chemokines responses (181, 184, 185). In addition to TLRs, significant evidence has been accumulated in recent years implicating a TLR9-independent mechanism for sensing Ad5 double-stranded DNA (dsDNA) genomes (195, 200, 201). Takaoka et al. showed that a dsDNA sensor called DNA-dependent activator of interferon regulatory factors (DAI) activates type I interferon in response to DNA viruses in L-929 cells, but subsequent studies suggested the presence of other cytoplasmic DNA sensor(s) (119, 202, 203). Ads have also been shown to activate the NALP3 inflammasome. The activation of the NALP3 inflammasome by Ad derived dsDNA leads to caspase-dependent activation of IL-1β and induction of pro-inflammatory cytokine and chemokine responses including elevations of IL-6, MIP-1β, IP-10, and MCP-1 (204). In addition, Ad mediated disruption of lysosomal membranes, and the release of cathepsin B into the cytoplasm, are required for Ad-induced NLRP3 inflammasome activation (205). Furthermore, Ad5 activation of NLRP3 also induced necrotic cell death, resulting in the release of the proinflammatory molecule HMGB1 (High-mobility group box 1 protein), a recently identified DAMP that mediates the response to infection, injury, and inflammation (205, 206). In addition, it has been demonstrated 34 that adenovirus virus-associated RNA (VA) , a dsRNA that inhibits interferon and RNAi sensing mechanisms, is no less recognized by RIG-I, a cytosolic pattern recognition receptor, and activates RIG-I downstream signaling, leading to the induction of type I IFNs (207). 1.7.2 Adaptive immune responses to Ad vector expressed transgenes Ad-based vectors have a potent ability to induce potent humoral, but more importantly, cellular (CTL) immune responses to expressed foreign antigens, and have therefore recently received much attention for use in a number of vaccine based applications (169, 208). Specifically, E1 deleted Ad5 vectors expressing the HIV-1 gag, pol and nef genes have been utilized in human trial subjects (209, 210). The results from the early-phase clinical trials demonstrated that the Ad5 vector-based vaccines elicited some of the most potent, HIV-1specific cellular immune responses in humans to date, however, the presence of pre-existing Ad5-specific neutralizing antibodies partially suppressed these responses (209, 210). Utilization of advanced generation Ad vectors have also recently been shown to allow for improved efficacy in several vaccine based applications (211-213). Specifically, [E1-,E2b-]Ad5 vectors were able to induce heightened antigen-specific T cell responses in mice and primates despite pre-existing Ad5 immunity (211, 212, 214). In contrast to E1 deleted Ad vectors, these types of Ad vaccines can show strong efficacy despite the existence of pre-existing Ad+ immunity, possibly by their avoidance of CD8 T cell responses directed to the Ad polymerase gene (211-215). [E1-, E2b-]Ad vectors, expressing tumor derived antigens were also able to induce beneficial, cytolytic T cell responses that promoted tumor regression in murine models (213). Based upon these improvements, a phase I/II clinical trial is currently underway, utilizing a CEA (Carcinoembryonic Antigen) expressing [E1-,E2b-]Ad5 vector in an attempt to safely 35 induce beneficial, CEA specific adaptive immune responses in patients (both Ad5 naive and Ad5 immune) bearing CEA expressing tumors. In summary, use of Adenovirus vectors for vaccine development is a promising approach in terms of inducing potent adaptive (cellular) immune responses to vaccine antigens. Harnessing the innate immune system is a valid strategy for shaping the adaptive immune responses to vaccine antigens. Several classes of vaccine adjuvants are currently under evaluation in both preclinical and clinical trials. However, their mechanisms of actions are not completely understood. Understanding the molecular mechanisms of vaccine adjuvants and further developments of Ad5 vaccine vectors to enhance their efficacy are now justified. Possibly a combination of Ad5 vector adjuvant activity and activation of a unique arm of the innate immune system may result in production of more efficacious Ad-based vaccine platforms. In this dissertation, we described a novel class of Ad5-based vaccine vectors that expresses the SLAM family of receptors adaptor molecule, EAT-2, and outline superior qualities relative to current generation rAd5-based vaccines. We also proposed to utilize the novel immunomodulatory protein “rEA” to enhance vaccines efficacy. Potentially in combination with other vaccine approaches, such as vectors- or DCs-based vaccines, to further enhance induction of antigen specific adaptive immune responses. 36 Chapter II Expression of the SLAM family of receptors adapter EAT-2 as a novel strategy for enhancing beneficial immune responses to vaccine antigens This chapter is the edited version of a research article that was published in the Journal of Immunology, Volume 186, Issue 2 (722-732), Jan 15, 2011. Authors: Aldhamen Y.A., Seregin S.S., Appledorn D.M. , Liu CJ, Schuldt N, Godbehere S, and Amalfitano A. 37 2.1 Introduction Development of an effective vaccine to prevent infections by the human immunodeficiency virus-1 (HIV-1) is an important goal. Most recently, a human clinical trial demonstrated that a prophylactic vaccine to HIV may indeed be possible (216). However, the results of that trial combined with recent results derived from the Merck® sponsored STEP trial, suggest that a more potent vaccine capable of inducing greater levels of antigen specific adaptive immune responses may demonstrate greater efficacy to prevent HIV infection (210, 217). Incorporation of adjuvants into vaccine formulations can improve the induction of antigen specific adaptive immune responses (218-220). Pro-active induction and/or harnessing of beneficial innate immune responses may be the mechanism underlying the effectiveness of certain adjuvants to significantly contribute to the ability of vaccines to generate adaptive immune responses (2, 108). Here, we wished to evaluate the potential of improving vaccination efficacy by presenting a target antigen while simultaneously activating novel arms of the mammalian immune system. While in previous studies we and others have explored the potential of modifying TLR dependent innate immune responses to facilitate improved efficacy of virus based vaccines (133, 221), in this study we set out to determine if targeted manipulation of the signaling lymphocytic activation molecule family of receptors (SLAM) pathway could also facilitate improved induction of HIV-1-Gag-specific, adaptive immune responses by vaccine platforms. In this chapter, I will provide a brief background about human immunodeficiency virus type I (HIV-1) biology, innate and adaptive immune responses to HIV-1, immune evasion mechanism of HIV-1, describe the current state of HIV-1-vaccine field and outline strategies for the development of an effective HIV-1 vaccine, and finally introduce a novel strategy for HIV-1 38 vaccine development by targeting the SLAM family of receptor signaling by EAT-2 adaptor protein. 2.1.1. Human immunodeficiency virus (HIV) and AIDS global epidemic HIV-1/ AIDS continue to be a significant health threat both in the United States and world-wide. AIDS was first recognized in 1981 and by 1983; HIV was identified to be the causative agent of this devastating disease. To date, more than 20 million people worldwide have died as a result of HIV-1infection. The global number of people living with HIV-1 was estimated to be 33.3 million in 2009 (222). HIV infection is associated with high morbidity and mortality contributing to 2 million deaths and 2.7 million being newly infected each year (222). Despite the intensive research in the past three decades, the AIDS epidemic is still spreading in an uncontrolled fashion across the globe, emphasizing the need to urgently develop effective vaccines, microbicides, and other preventative strategies to blunt the progress of the epidemic and to stop HIV-1 transmission. Since its discovery, the origin of HIV virus remains a topic of great interest for both the public and scientific community. HIV belongs to the lentiviral genus of the family retroviridae. Phylogenetic analysis of the lentiviral lineage has revealed that HIV was originated from multiple zoonotic transmissions of simian immunodeficiency virus (SIV) from non-human primates (NHP) into humans in West and Central Africa (223). More than 40 different nonhuman primate species harbor SIV infections. Thus, several independent zoonotic transmission events from NHP to humans have generated several HIV lineages; HIV type 1 (HIV-1) groups M (main group; has nine subtypes), O (outlier group), N (non-M/ non-O), P (proposed groups), and HIV type 2 (HIV-2) groups A-H (224). Each group of HIV causes a 39 relatively different epidemic. HIV-1 group M is responsible for the global HIV infection (approximately 33 million infected individuals), group O causes tens of thousands of infections in West and Central Africa, group N has been found in few people in Cameroon, and group P was recently identified in two individuals originating from Cameroon (224). HIV-2, on the other hand, causes a relatively attenuated pathogenicity in human. 2.1.2. HIV-1 genetic diversity and its impact for HIV-1 control Similar to most RNA viruses, the HIV-1 virus demonstrates an enormous genetic variability and rapid evolution rate due to the poor fidelity of reverse transcriptase and a lack of proofreading machinery (225). The estimated mutation rate for HIV-1 is 5 × 10 -5 mutations/site/generation (225). HIV-1 mutation and recombination result in the rapid generation of genetically diverse viral populations within each infected individual (226). Several full-length genome sequencing studies have revealed that recombination between strains is a much more powerful and relevant force in shaping HIV-1 evolutionary patterns than individual point mutation frequency (227). Further analysis revealed that recombination does not appear to be limited by sequence similarity, as recombination has occurred between strains from different HIV-1 groups (groups M and O) as well as between and within group M subtypes (228). As a result of this rapid evolution and diversification of the HIV-1 virus, the genetic diversity within the HIV-1 group M envelope, Env, is estimated to be as high as 35% and recombinants are currently estimated to be responsible for at least 20% of HIV-1 infections worldwide (229). This issue represents one of the most challenging factors for controlling disease progression, in designing effective anti-retroviral therapies, and in manufacturing effective therapeutic and preventative HIV-1 vaccine (230). 40 2.1.3. Immune response to HIV-1 and immune evasion mechanisms 2.1.3.1 Innate immune responses to HIV-1: It is well established that HIV-1 infection is associated with the modulation and dysregulation of the immune system (231). Adaptive immune responses occur late in HIV-1 infection, with T cell responses appearing 1-2 weeks after initial infection and neutralizing antibodies appearing 3 months after infection (232), emphasizing a critical role for the innate immune system in early anti-HIV-1 responses. The role of the innate immune system in HIV-1 infection has been studied extensively. The innate immune response to HIV-1 includes a variety of cellular, extracellular, and intracellular components that initiate diverse set of signaling pathways that restrict growth and replication of HIV-1 (233). The innate immune cells that have been shown to be involved in protection from HIV-1 infection include: Langerhans cells in vaginal and foreskin epithelia (234), γδ T cells in rectal and vaginal epithelia, and macrophage, DCs, and NK cells in the subepithelial tissues (235). DCs and NK cells have central roles in antiviral immunity by shaping the quality of the adaptive immune response to viruses and by mediating direct antiviral responses (236). HIV-1 infection has been shown to dysregulate and reduce DCs number in the blood, a phenomenon that correlates with increased viral load and disease progression (237). The interaction between HIV-1 and DCs is complex and not yet fully understood. Several DC-expressed surface receptors (for example, CCR5, DC-SIGN, and CXCR4) have been shown to be target for binding and internalization of HIV-1 (238). Commonly, recognition of single stranded RNA by TLR-7 in pDCs, activates IRF signaling pathways that results in, production of type I interferons (as well as other cytokines), stimulation of cDCs activities, and directly enhancing NK cell responses (239). HIV-1 infection does not induce cDCs maturation through TLR activation. The 41 mechanism of this inhibition is not fully understood, however, low HIV-1 viral replication within cDCs, the interactions between HIV-1 and C-type lectins (which abrogate TLR responsiveness), and prevention of autophagy-mediated viral degradation, are all proposed mechanisms for this inhibitory process (240). In addition, it has been shown that type I IFN production from pDCs can facilitate HIV-1 infection by enhancing HIV-1 replication. For example, IFNα production + from pDCs has been shown to up-regulate CD4 T cell expression of the pro-apoptotic molecules, such as TNF-related apoptosis-inducing ligand (TRAIL), death receptor 5 (DR5), and + FAS, leading to a progressive CD4 T cell loss (241). Furthermore, it has been shown that HIV1-exposed pDCs can promote the development of Treg cells, which prevent the induction of effector T cell responses by inhibiting the maturation of cDCs (242). Moreover, as DCs can regulate NK cell activation, dysregulation of DCs have been shown to contribute to the dysregulated NK cell function commonly observed in HIV-1 infected individuals. NK cells prevent the early spread of viruses by producing cytokines and directly killing infected cells (243). NK cells interact with DCs to shape the magnitude and quality of the adaptive immune response (244). NK cells responses to viral infection is controlled by several inhibitory and activating receptors (245). For example, lack of the MHC-I expression (which binds NK cell inhibitory receptor) on viral infected cells and/ or expression of ligand for NK cell activation receptors, are two mechanisms that have been described to regulate NK cell responses during viral infections (243). To date, no specific NK cell receptors that directly recognize HIV1-infected cells have been identified (236). Several lines of investigations have shown that HIV1 virus utilizes several mechanisms in order to evade NK cell responses. For example, it has been shown that the HIV-1 Nef (negative factor) that selectively down-regulates the expression of 42 HLA-A and HLA-B, but not HLA-C or HLA-E, allows HIV-1 to evade CTL responses, responses that are largely directed against HLA-A and HLA-B-restricted epitopes (246). Whereas, preserving HLA-C and/ or HLA-E (which interact with NK cell inhibitory receptors) in the surface of HIV-1 infected cells, prevent efficient killing by NK cells (247). Furthermore, it has been shown that HIV-1 Nef impairs NK cell activity by down-regulating the expression of ligands (MICA, ULBP1, and ULBP2) for the NKG2D NK cell activation receptor (248). During early stages of HIV-1 infection, several extracellular components of the innate immune system, inclusive of pro-inflammatory cytokines and chemokines, have been shown to increase in the plasma of HIV infected individuals. For example, the plasma levels of type I IFN, TNFα, IL-6, MCP-1, IFNγ, and IL-10 have all been found to be upregulated at various stages following HIV-1 infection (249). These innate immune responses however have not been associated with control of viremia, indicating that the rapid activation of systemic cytokine cascade is not a prerequisite for viral clearance. Rather, it has been suggested that the induction of these pro-inflammatory mediators may promote HIV-1 replication and suppress the antiviral effect of type I IFNs (249). In addition, the CC chemokines MIP-1α (CCL3), MIP-1β (CCL4), and RANTES (CCL5) produced from activated macrophages, DCs, T-, NK-, and γδ T-cells have all been shown to play a role during HIV-1 infection. By binding and down-modulation of the CCR5 HIV-1 co-receptor, these CC chemokines have been shown to prevent HIV-1 infection in vitro and SIV infection in vivo (235). It has been shown that induction of these 3 CC chemokines following vaccination of rhesus macaques with SIVgp120 and p27, inversely correlated with proportion of cells expressing CCR5 co-receptor (250), suggesting that vaccination strategies that up-regulate these CC chemokines may prevent HIV-1 infection. 43 Several intracellular molecules have also been found to play critical roles during HIV-1 infection. For example, the type I IFN induced nucleic acid editing enzyme, APOBEC3G, has been shown to prevent HIV-1 viral replication (251). APOBEC3G is a cytosine deaminase that converts cytidine to uridine in single stranded proviral DNA, which have been found to induce hyper-mutation of the HIV-1 genome, rendering them non-functional and inhibiting viral replication (252). This innate mechanism of resistance to HIV-1 infection has been found to be counteracted by HIV-1 viral infectivity factor (vif) which prevents incorporation of APOBEC3G into the virion and rapidly induces APOBEC3G ubiquitination and proteasomal degradation (253). Induction of APOBEC3G in immune cells may represent a novel strategy for the + development of next generation HIV-1 vaccines. APOBEC3G upregulation in DCs and CD4 T cells has been found to be mediated by several mechanism, including ligation of CCR5 (via MIP1α) and CD40 (via CD40L), and by the 70-kDa heat shock protein (HSP70) (254). Recently, APOBEC3G has been shown to be induced in CD4+ memory T cells of rhesus macaques following immunization with SIVgp120 and CCR5 peptides linked to HSP70 (255). Furthermore, mucosal challenge with SIVmac251 showed a significant increase in APOBEC3G + + mRNA in the CD4 CCR5 circulating memory T cells and the draining iliac lymph node cells in the immunized uninfected macaques, suggesting a protective effect exerted by APOBEC3G (255). In addition to APOBEC3G, the family of tripartite motif (TRIM) proteins is another example if type I IFN induced genes that have also been shown to restrict HIV-1 replication. In rhesus macaques, TRIM-1α has been shown to block the early replication steps of HIV-1 and other retroviruses by targeting the viral capsid (CA) protein for degradation (256). Moreover, it has also been shown that the type I IFN induced protein, Tetherin (BST-2, also called CD317), 44 can also restrict HIV-1 by preventing the release of newly synthesized virions from HIV-1 infected cells, a mechanism that is counteracted by HIV-1 vpu protein, which targets the transmembrane domain of tetherin for ubiquitin dependent proteasomal or lysosomal degradation (257). HIV-1 virus has also developed several mechanisms that prevent type I IFN activation in general. For example, HIV-1 vpr and vif proteins have been shown to target IRF-3 for degradation in T cells, a mechanism that results in inhibition of the antiviral response and IFNβ synthesis (258). Furthermore, the HIV-1 encoded protease was also found to play a role in the inhibition of type I IFN by enhancing IRF-3 phosphorylation and thus preventing the induction of ISG (259). Thus, despite these potent innate effector mechanisms, HIV-1 developed several mechanisms to evade these innate responses. These facts indicate that the innate immune response to HIV-1 is complex and represent a novel mechanism in preventing HIV-1 transmission or controlling virus replication until the development of an effective adaptive immune response. It also indicates that, harnessing the innate immunity could be a valid strategy for the development of future HIV-1 vaccines. 2.1.3.2. Adaptive immune responses to HIV-1: HIV-1 is predominantly transmitted through mucosal tissues, targeting and destroying + + 50% of CD4 CCR5 T cells within two weeks following the infection, setting the base for progressive immunodeficiency (260). As mentioned above, the immune system recognizes and initially controls HIV-1 replication but is incapable of fully eradicating the virus (232). HIV-1+ specific CD8 cytotoxic T cell responses have been detected at early stages following control of primary viremia (261), implicating an important role for CTL responses in immune control of viral replication. Several reports have also shown that cellular immune responses to HIV-1 are a 45 driving force for HIV-1 viral evolution (232), indicating a selective pressure by these cellular + immune responses. The Gag-specific CD8 T cell responses have been shown to inversely correlate with HIV-1 RNA levels in chronically infected individuals (262). In addition, genetic association studies have demonstrated that specific human leukocyte antigen (HLA) alleles are closely associated with HIV-1 RNA levels (263). Moreover, it has been shown that HLAmediated escape mutations correlate with sites of inter- and intra-HIV-1 subtype variability + (264). Furthermore, depletion of CD8 T cells abrogated the control of SIV replication during both acute and chronic infection (265). The important role of cellular immune responses to HIV-1 has been further confirmed by several pre-clinical and clinical vaccine studies (208). For example, several reports on SIV infected rhesus monkeys have shown that vaccine strategies that elicit potent cellular immune responses can reduce the setpoint viral load in specific SIV challenge models (266, 267). It is + worth mentioning here that the CD8 T cell, both natural or vaccine-elicited, responses are not sufficient to completely prevent or eliminate the viral infection, however, by potentially lowering the viral load set points, these cellular immune responses may help in minimizing both the rate of disease progression in infected individuals and the transmission of the infection to other individuals (268). The role of humoral immune responses to HIV-1 has also been studied extensively, and is again in the forefront based primarily upon results using HIV vaccines using the env gene to generate neutralizing antibody responses that can be protective to some vaccinees (269). Antibody responses of different isotypes to HIV-1 proteins env, gag, and pol have been found to be elicited early following HIV-1 infection. Several reports have indicated that the initially 46 induced antibody response to HIV-1 is ineffective in controlling virus replication during acute HIV-1 infection (270). HIV-1 specific IgM and IgG antibody responses to the envelope glycoprotein (Env) (anti-pg41 (appeared at 13 day) and anti-gp120 (appeared at 28 day)) have been detected in HIV-1 infected individuals; however, these responses are non-neutralizing and have been shown to have a limited impact on acute-phase viremia (270). In addition, IgM and IgG antibody responses to gag (appear at 18 day) and integrase (appear at 53 day) have also been detected, however these responses have been found to have limited impact on viral RNA level (231). Broadly reactive neutralizing antibody responses to HIV-1 Env have been suggested to be the most important correlate of protection against HIV-1 infection. Recent studies by Barouch et al group revealed that virus vaccine-elicited neutralizing antibody responses to Env protect against acquisition of fully heterologous, neutralization-resistant SIV challenges in rhesus monkeys (267), suggesting that neutralizing antibody responses may contribute significantly to HIV-1 control and prevention. However, in chronic infection, neutralizing antibody responses to Env are rarely generated and have been shown to appear several months (12 weeks) after the HIV-1 infection (270). In addition, these HIV-1-specific anti-envelope responses were primarily directed to gp120 and found to be predominantly IgG1 subtype, indicating that at chronic HIV-1 infection, the T helper responses were skewed to Th2 (271). Further, IgG2, IgG3, and IgG4 antibodies against gp120 were also detected, but are generally less often and not associated with control of viremia. Furthermore, the range of epitopes targeted by these initial neutralizing antibodies is very narrow allowing rapid viral escape (272). Several studies have shown that Env neutralizing antibodies are directed against the monomeric gp120, rather than targeting the intact HIV-1 Env trimer on the virion surface (273). In addition, the conserved HIV-1 CD4-binding site, the chemokine receptor binding site, and key epitopes in the membrane proximal external 47 region (MPER) of gp41, are all targets for broadly reactive neutralizing antibodies; however, these sites were found to be hidden or mutated and only exposed during receptor binding (232, 272). Furthermore, mucosal HIV-1-specific neutralizing IgA antibodies of unknown specificity have been detected in genital secretion of high-risk HIV-1 uninfected sex workers and found to correlate with subsequent protection from HIV-1 acquisition (274). Thus, development of vaccine platforms that induce broadly reactive neutralizing antibodies that target the CD4binding site, glycan on the surface of pg120, and the MPER region of the gp41, is a primary area of research for future antibody-based HIV-1 vaccines. Several studies in rhesus monkey have shown that administration of high doses of broadly reactive monoclonal antibodies can block transmission of SIV (275). More recently, the results from the RV144 phase III “Thai” trial (four priming injections of a recombinant canarypox virus expressing gp120, gag and protease as priming vaccination (ALVAC-HIV) followed by a two booster injections of a recombinant glycoprotein 120 subunit vaccine (AIDSVAX B/E)) suggested that a prophylactic HIV-1 vaccine is possible and protection from HIV-1 infection can be achieved with vaccine strategies that can induce more potent T and B cells responses to HIV-1-derived antigens, with gp120 being primarily implicated in this study (269). Moreover, the results from this trial suggest that the vaccine induces a weakly protective effect against viral acquisition but not viral control after infection (276), suggesting a role for the elicited binding antibodies to gp120 protein. 2.1.4. HIV-1 vaccine trials The best hope of controlling HIV-1 epidemic is through the development of a successful prophylactic vaccine. Previously performed HIV-1 vaccine studies have relied primarily in the 48 induction of either neutralizing antibody or T cell-mediated immune responses, however, most of these responses have largely failed to protect from HIV-1 infections. More recently, it has been shown that vaccine regimens that can induce both cellular and humoral immune responses may prevent from HIV-1 infection in human (269). Since Edward Jenner’s discovery of vaccination to prevent smallpox, elicitation of pathogen-specific antibody responses was regarded as the best correlate of protection. Utilizing this concept, two HIV-1 vaccine efficacy studies were performed to date. The first study utilized purified monomeric envelope glycoprotein (Env gp120) subunit vaccine in order to generate HIV-1-specific antibody responses. The result from this trial revealed that antibody responses induced by this vaccine were unable to neutralize primary virus isolates and to protect from HIV1 infection (277). Another two phase III studies known as AIDSVAX (VAX003 and VAX004) (conducted by VaxGen in 2003) were performed to evaluate the protective efficacy of alum adjuvanted Env gp120 protein. The results of these two studies have also indicated that rgp120 vaccination does not provide protection against HIV-1 infection in human (clinical isolates were highly resistant to neutralization and the vast majority of antibodies induced by vaccination with monomeric Env proteins were directed to decoy epitopes with no neutralization abilities) (278), confirming the need to develop more potent, broadly reactive, neutralizing antibody responses by HIV-1 vaccines (279). The induction of potent cell-mediated immune responses against HIV-1 has been the focus in the past few years. Several reports have demonstrated that induction of potent CTL responses is the primary correlate of protection against viral infection (280). In addition, the result from early studies in NHP confirmed the importance of CTL responses in controlling HIV1 infection (265). Moreover, CTL responses (predominantly gag-specific responses) in a group 49 of HIV-1 infected patients who control viral replication without therapy, called “elite controllers”, indicated a direct correlation between strong CTL responses and control of viremia (281), justifying the development of vaccination approaches that elicit potent CTL responses. Studies in NHPs challenged with a hybrid SHIV virus utilizing different recombinant adenovirus platforms supported a rAd5-based HIV-1 vaccine clinical trial (known as STEP® trail; HVTN 502) performed by Merck Research Laboratories. Recombinant Ad5 vaccines expressing several HIV-1 antigens (HIV-1 clade B Gag, Pol and Nef) clade B Gag were utilized in a homologous prime-boost regimen at months 0, 1, and 6. The results from the trial revealed that cellular immune responses specific for the HIV-1 antigens were induced in the majority (73%) of vaccinated individuals (209, 217). The inductions of these cellular immune responses were completely blunted in individuals with pre-existing Ad5-specific immunity. The STEP study was terminated in 2007 due to lack of efficacy, as no evidence of vaccine induced protection (preventing acquisition of infection or facilitating control of HIV-1 replication postinfection) was observed in vaccinees (210). Similar clinical trial conducted in Thailand, known as Phambili (HVTN 503), was also terminated because it utilized the same rAd5 vaccine vectors in a population with higher levels of pre-existing Ad5-specific immunity. While it was initially thought that pre-existing immunity to Ad might enhance HIV infection rates in the STEP vaccinees, subsequent studies have noted that circumcision rates and/or other confounding factors likely contributed to the skewed infection rates noted between placebo and Ad vaccinated, Ad immune participants in the trial (282-284). The negative results from antibody-based and the Merck rAd5-based trials led to development of different strategies aiming to develop more potent humoral and cellular immune responses to HIV-1 antigens. Heterologous prime-boost vaccination strategy has been shown to 50 provide higher magnitude (and quality) B- and T-cell responses, compared to homologous prime-boost vaccination approaches. More recently, it has been shown that, a heterologous prime-boost vaccination against HIV-1 might be an effective strategy to prevent HIV-1 infection in humans. The results from the clinical trial, RV144, showed 31% reduction in infection acquisition, but not control of viremia, in vaccinees compared to controls (269). The ALVAC prime-gp120 boost vaccine regimen induced strong transient Env-specific antibody responses + + and Env-specific CD4 T cell responses, but no significant HIV-specific CD8 T cells responses. This strongly suggests a critical role for humoral immune responses in preventing + viral acquisition and potentially the need to elicit more potent CD8 T cells responses to control viral replication post infection in more vaccinees. Importantly, similar protection levels were also observed in NHP studies. Recent heterologous prime-boost (rAd26 prime/ modified vaccinia Ankara (MVA) boost) studies in NHPs have shown a critical role for the elicited neutralizing antibody responses to Env to protect against acquisition of fully heterologous, neutralization-resistant SIV challenges in rhesus monkeys (267). In another study, a prime-boost (A plasmid DNA prime/ rAd5 boost) vaccine regimen was evaluated for ability to protect monkeys from infection by SIVmac251 or SIVsmE660 isolates after repeat intrarectal challenges. The prime-boost vaccination protected 50% of vaccinated monkeys from acquisition of SIVsmE660, but had a minimal effect on viral loads of successfully infected monkeys. Notably, low levels of neutralizing antibodies and an + envelope-specific CD4 T cell response were associated with vaccine protection in these monkeys (285). These studies begin to elucidate the mechanisms of vaccine protection against 51 HIV-1 and highlight the need to develop vaccine strategies capable of eliciting more potent humoral and CTL immune responses in future trials of HIV-1 vaccines. The previous data described above laid the foundation for the original body of work described herein. While it has become increasingly accepted that use of the rAd5 vaccine vectors elicits potent antibody and cellular immune responses to specific antigens as compared to immunizations using other vaccine platforms, the results from STEP trial suggest that a more potent vaccine capable of inducing greater levels of antigen specific adaptive immune responses may demonstrate greater efficacy to prevent HIV-1 infection. The work in this chapter attempts to describe the development of a novel rAd5-based HIV-1 vaccine approach that targets the SLAM family of receptors signaling in innate immune cells by expressing an adaptor molecule, known as EAT-2, identified to be critical for regulating signal transduction and proinflammatory cytokines responses downstream of SLAM receptors in innate immune cells. The SLAM family of receptors currently comprise six distinct members, respectively named SLAM (CD150), 2B4 (CD244), Ly9, CD84, NTB-A (natural killer, T and B cell antigen; Ly108 in the mouse) and CRACC (CD2-like receptor activating cytotoxic cells) (286). These receptors are expressed mainly in cells of the hematopoietic lineages, inclusive of innate and adaptive immune cells (149, 287). All SLAM members except 2B4, (which interacts with CD48) are self ligands, and can be triggered by homophilic interactions through their respective extracellular domains to initiate intra-cellular signaling via recruitment of specific adaptors (287290). The SLAM-associated protein (SAP) family of adaptors includes three members named SAP, EAT-2, and EAT-2-related transducer (ERT; ERT is however a non-functional pseudogene in humans). These adaptors associate with phosphorylated tyrosine-based motifs (‘immunoreceptor tyrosine based switch motifs’ (ITSM)) in the cytoplasmic domains of SLAM 52 receptors with high affinity and specificity. Once bound, these adaptors either augment or inhibit SLAM induced intracellular signaling in a variety of immune cells (286). EAT-2 and ERT transduce SLAM initiated signals via phosphorylation of tyrosine residues directly located in their short carboxyl-terminal tails (165). In contrast, the SAP adaptor regulates SLAM signaling by recruiting the protein tyrosine kinase FynT. Various reports indicate a possible role for the binding of SLAM (CD150) receptor in the immunological synapse, whereby SLAM receptor activation acts as a costimulatory molecule facilitating the activation of DCs and macrophages. For example, in CD40L-activated human DCs, antibody-mediated ligation of SLAM receptor augmented the secretion of proinflammatory cytokines such as IL-12 and IL-8, but not IL-10 (291). Furthermore, the SLAM receptor was also found to play a role in the production of IL-6 and IL-12 by mouse peritoneal macrophages (292). In addition, macrophages derived from SLAM-deficient mice show a marked reduction in secretion of IL-12, TNF, and nitric oxide (292). Since EAT-2 is the only known SLAM-associated adaptor protein expressed in DCs and macrophages, it has been proposed that EAT-2 facilitates SLAM dependent proinflammatory cytokine expression in these cell types (290). Relative to vaccine augmentation strategies, we note that the TLR adaptor MyD88 also facilitates cytokine gene expression during TLR dependent signaling, and that over-expression of MyD88 from DNA based vaccines facilitated the induction of antigen specific adaptive immune responses by the vaccine platform (293, 294). Based upon these facts, we hypothesized that expression of EAT-2 from a vaccine platform could also augment innate immune responses, potentially resulting in improved APC functions in vivo and consequently improvement in the induction of adaptive immune response generated against a co-expressed target antigen. In this 53 study, we confirmed that an adenovirus based vaccine expressing the SLAM adaptor molecule EAT-2 facilitates the induction of several arms of the innate immune system, and that these inductions positively correlate with an improved ability of the vaccine to induce stronger cellular immune responses to a co-expressed antigen. Our results highlight for the first time the use of a SLAM immune regulatory pathway component to improve vaccination efficacy. 54 2.2. Results EAT-2-expressing Ad vectors enhance Ad vector induced innate immune responses in vivo. We constructed an E1 and E3 deleted ([E1-])Ad vector specifically designed to express the murine homologue of the SLAM adaptor protein EAT-2. These EAT-2 expressing Ad vectors were fully viable, grew to high titers, and were purified and quantified exactly as done for conventional [E1-]Ad vaccines (184). To analyze the maximal impact that Ad-EAT2 expression might have upon innate immune responses, we administered Ad5-EAT2 or Ad-Null control into C57BL/6 mice and measured the levels of cytokines and chemokines by utilizing 23-plex multiplex based assay in the plasma at 3, 6, 10, 24, and 48 hours post injection (hpi). We targeted these time points to evaluate the kinetics of EAT-2 effects on the induction of pro-inflammatory cytokines and chemokines occurs after Ad5-EAT2 vector administration. Administration of the Ad5-EAT2 vector into C57BL/6 mice resulted in induction of significantly higher plasma levels of, IL-1a, IL-5, IL-12p70, IFNγ, G-CSF, and GM-CSF as directly compared to mice identically treated with an Ad-Null control vector (Figure 1a). Interestingly, the levels of EOTAXIN, IL-10, and KC were significantly reduced in Ad5-EAT2 injected mice compared to Ad-Null injected controls (Figure 1b). The levels of IL-1β, IL-12p40, IL-6, IL-9, MCP-1, MIP-1α, MIP-1β, and RANTES were also significantly induced by Ad5-EAT2 vectors; however, these levels were not statistically different between Ad5-EAT2 and Ad-Null injected mice (data not shown). We also measured the levels of IL-3 and IL-4, however, no significant inductions were observed in mice injected with both Ad-EAT-2 and Ad-Null vectors (data not shown). 55 Figure 1: Systemic administration of EAT-2 expressing adenovirus vector induces cytokine and chemokines responses. C57BL/6 mice (n=4) were either mock injected, or intravenously injected with 7.5×10 10 vps of either Ad-Null or Ad-EAT2 vectors. Plasma was harvested at 3, 6, 10, 24, and 48 hours after virus injection. Cytokine induction was evaluated using a multiplexed bead array based quantitative system. The bars represent mean ± SD. Statistical analysis was completed using One Way ANOVA with a Student-Newman-Keuls post-hoc test, p<0.05 was deemed a statistically significant difference. ** denotes p<0. 01, *** denotes p<0. 001- Statistically different from mock injected animals). 56 Figure 1: Systemic administration of EAT-2 expressing adenovirus vector induces cytokine and chemokines responses. 57 To first determine what innate immune cell types may be transduced by the Ad vectors expressing EAT2, (and potentially be responsible for the enhanced cytokine responses noted in Fig 1), we analyzed Ad vector transduction of several important classes of innate immune cells found in the spleen by flow cytometry. Utilizing an Ad vector expressing a tracking antigen (GFP) we confirmed that Ad vectors can transduce dendritic cells, NK cells, as well low levels of T cells upon administration into mice (Figure 2a-d). Interestingly, we found that the highest + + subset of DCs that were transduced by Ad-GFP vectors were CD11c , CD8α , a distinct subset of dendritic cells shown to play a critical role in shaping adaptive immune responses after vaccination (190) (Figure 2a). Additionally, we confirmed that after identical administrations of the Ad-EAT2 vector, EAT-2 gene expression was occurring in spleen cells as well (Figure 2e). Figure 2: Transduction efficiency of innate immune cells by Adenovirus vectors expressing transgenes. C57BL/6 mice (n=3) were either mock injected, or intravenously injected with 7.5 × 10 10 vps of Ad-GFP. Splenocytes were harvested 6 hours post-injection and GFP expressing cells were detected and identified by gating on FITC+ cells using a LSR-II flow cytometer. (A) + + + Ad-GFP Transduced CD11c CD8α dendritic cells. (B) Ad-GFP transduced CD11c CD8α dendritic cells. (C) Ad-GFP transduced CD3- NK1.1+ cells. (D) Ad-GFP transduced CD3 + - + CD8 T cells. (E) Quantitative RT-PCR for EAT-2 transcript derived from the spleen of Mock, Ad-GFP, or Ad-EAT2 injected C57BL/6 mice. The bars represent mean ± SD. Statistical analysis was completed using student t-test, p<0.05 was deemed a statistically significant difference. * denotes p<0. 05- Statistically different from mock injected animals. 58 Figure 2: Transduction efficiency of innate immune cells by Adenovirus vectors expressing transgenes. 59 It is widely appreciated that Ads enhance innate immune cell effector functions (295, 296). To additionally evaluate the phenotype of immune cells following exposure or transduction by the Ad vector expressing EAT-2, we analyzed the expression of the lymphocyte activation marker CD69 as well as IFN-γ production in various immune cells shortly after administration of Ad-EAT2 into C57BL/6 mice. Our results confirmed that Ads, in general, significantly induce a rapid activation of NK and NKT cells in both PBMCs and spleens, as confirmed by the presence of increased percentages of CD69 expressing NK and NKT cells in Ad-GFP treated mice (Figure 3a and b). Specifically, injection of Ad-EAT2 induced significantly higher numbers of CD69 expressing NK and NKT cells, both in PBMCs and splenocytes at 6 hpi, as compared to the AdGFP control vector (Figure 3a and b). Figure 3: Ad-EAT2-mediated activation of innate and adaptive immune cells in vivo. C57BL/6 mice (n=4) were either mock injected, or intravenously injected with 7.5 × 10 10 vps of either Ad-GFP or Ad-EAT2. CD69 expression by PBMCs (A) and splenocytes (B) derived NK, + + + - NKT, CD3 CD8 , CD3 CD8 , and B cells was evaluated 6 hours after virus injection. PBMCs and Splenocytes were harvested, stained and sorted on a LSRII flow cytometer. The bars represent mean ± SD. Statistical analysis was completed using One Way ANOVA with a student- Newman-Keuls post-hoc test, p<0.05 was deemed a statistically significant difference. * denotes p<0.05, ** denotes p<0. 01, *** denotes p<0. 001, # denotes p<0. 001- Statistically different from mock injected animals. 60 Figure 3: Ad-EAT2-mediated activation of innate and adaptive immune cells in vivo. 61 At 48 hpi, CD69 expression on NK cells remained significantly higher (p<0. 05) only in PBMCs derived from Ad-EAT2 injected mice as compared to Ad-GFP injected mice (Figure 4a). All other differences previously noted at 6 hpi had dissipated by 48 hpi (Figure 4c), suggesting that expression of EAT-2 by Ad vaccines transiently improves the induction of these responses. Figure 4: Ad-EAT2-mediated activation of innate and adaptive immune cells in vivo. C57BL/6 mice (n=4) were either mock injected, or intravenously injected with 7.5 × 10 10 vps of either Ad-GFP or Ad-EAT2. CD69 expression by PBMCs (a and b) and splenocyte (c and d) + + + - derived NK, NKT, CD3 CD8 T cells, CD3 CD8 T cells, and B cells was evaluated 48h after virus injection. PBMCs and Splenocytes were harvested, stained and sorted on a LSRII flow cytometer. The bars represent mean ± SD. Statistical analysis was completed using One Way ANOVA with a student- Newman-Keuls post-hoc test, p<0.05 was deemed a statistically significant difference. * denotes p<0.05, ** denotes p<0. 01, *** denotes p<0. 001- Statistically different from mock injected animals. 62 Figure 4: Ad-EAT2-mediated activation of innate and adaptive immune cells in vivo. 63 Treatment with either Ad-EAT2 or Ad-GFP induced significantly elevated numbers of IFNγ+ NK cells at both 6 and 48 hpi (p<0. 001 and p<0. 05, respectively). However, no significant differences were observed between the control and experimental viruses, suggesting that overexpression of EAT2 cannot induce additional inductions of IFNγ relative to the ability of the Ad vector itself (Figure 5). Figure 5: IFNγ production from NK cells 6 and 48 hours after Ads injection. C57BL/6 mice (n=4) were either mock injected, or intravenously injected with 7.5× 10 10 vps of either Ad-GFP or Ad-EAT2 for 6 hpi (a) or 48 hpi (b). Splenocytes were harvested and incubated at 37°C for 5 hours in the presence of Golgi plug. IFNγ intracellular staining was performed and cells were sorted on a LSRII flow cytometer. The bars represent mean ± SD. Statistical analysis was completed using One Way ANOVA with a student- Newman-Keuls post-hoc test, p<0.05 was deemed a statistically significant difference. *denotes p<0.05, *** denotes p<0. 001- Statistically different from mock injected animals. 64 Figure 5: IFNγ production from NK cells 6 and 48 hours after Ads injection. 65 Since the SLAM family of receptors are expressed in various innate and adaptive immune cells (149) and the activation of T cell and/or B cells can be initiated or accentuated by innate immune system activation , we sought to analyze adaptive immune cell responses shortly after administration of Ad-EAT2. Our result confirmed that Ad vector administration in and of + + + - itself induces a rapid activation of CD3 CD8 T-cells CD3 CD8 T-cells, and B cells, Ad dependent responses that can all be further accentuated with expression of EAT-2. For example, injection of Ad-EAT2 resulted in significantly higher numbers of splenic CD69 expressing + + + - CD3 CD8 T cells (p<0. 01), CD3 CD8 T cells (p<0. 01), and B cells (p<0. 001) at 6 hpi (Figure 6b) as compared to the numbers of these cells being induced by the control Ad vector. Figure 6: Ad-EAT2-mediated activation of innate and adaptive immune cells in vivo. C57BL/6 mice (n=4) were either mock injected, or intravenously injected with 7.5 × 10 10 vps of either Ad-GFP or Ad-EAT2. CD69 expression by PBMCs (a) and splenocytes (b) derived NK, + + + - NKT, CD3 CD8 , CD3 CD8 , and B cells was evaluated 6 hours after virus injection. PBMCs and Splenocytes were harvested, stained and sorted on a LSRII flow cytometer. The bars represent mean ± SD. Statistical analysis was completed using One Way ANOVA with a student- Newman-Keuls post-hoc test, p<0.05 was deemed a statistically significant difference. * denotes p<0. 05, ** denotes p<0. 01, *** denotes p<0. 001, # denotes p<0. 001- Statistically different from mock injected animals. 66 Figure 6: Ad-EAT2-mediated activation of innate and adaptive immune cells in vivo. 67 + + + - At 48 hpi, the percentage of splenic CD69 expressing CD3 CD8 , CD3 CD8 T cells, and B cells derived from Ad-EAT2 and Ad-GFP injected mice remained significantly higher (p<0. 001) over mock injected mice; however, no statistical differences were observed between the control and experimental Ad viruses at this time point (Figure 4d). We also evaluated the percentage of CD69 expressing cells in PBMCs from the same animals. At 6 hpi, we observed a significantly (p<0. 05) higher percentage of CD69 expressing total lymphocytes [as defined previously] in Ad-EAT2 injected mice as compared to Ad-GFP injected mice. When analyzing specific lymphocyte subsets, we observed a significant increase in the percentage of CD69 + - expressing CD3 CD8 T cells isolated from Ad-EAT2 injected mice as compared to the percentage of identical cells isolated from Ad-GFP injected mice (p<0. 05) (Fig 6a). Higher + - activation of CD3 CD8 T cells and B cells were observed in PBMCs derived from Ad-EAT2 injected mice as compared to the mock infected mice, however no statistically significant differences in activation levels were observed between the control and experimental Ads in these cell types (Figure 6a). By 48 hpi in PBMCs any statistically significant differences between control and experimental Ad vector injected animals had resolved (Figure 4b). Simultaneous administration of antigens with adjuvants can stimulate the innate immune system to significantly improve the adaptive immune responses to the antigenic target (143, 297). To investigate whether the enhanced innate immune responses promoted by Ad mediated expression of EAT-2 could influence the adaptive immune responses to a co-administered antigen, we immunized Balb/c or C57BL/6 mice with both an Ad-based vector expressing the HIV-1 clade B Gag protein (HXB2) along with the Ad-EAT2 vector, and a control vector (Ad-GFP). We performed initial dose curve studies to identify the lowest dose of Ad-HIV/Gag that generated 68 detectable Gag-specific cellular immune responses in the two distinct strains of mice. As a result, 6 8 we identified an Ad-HIV/Gag dose of 5 x10 vps/mouse for Balb/c mice, and 5 x10 vps for C57BL/6 mice as the most relevant experimental doses for these studies (data not shown). Six 6 days after Balb/c mice were intramuscularly injected with 5x10 of Ad-HIV/Gag mixed with equivalent amounts of either the control Ad-GFP vector, or Ad-EAT2, we were able to detect + heightened Gag specific, tetramer-positive CD8 T cells in PBMCs derived from mice coimmunized with Ad-HIV/Gag + Ad-EAT2 as compared to Ad-HIV/Gag+ Ad-GFP coimmunized mice (Figure 7a ). At 14 dpi , PBMCs (p<0. 05) and splenocytes (p<0. 05) derived from mice co-immunized with Ad-HIV/Gag + Ad-EAT2 contained higher numbers of Gagspecific tetramer-positive CD8+ T cells as compared to the respective cell populations isolated from control mice (Figure 7b and c). Figure 7: HIV-Gag specific cellular immune responses elicited by Ad-HIV/Gag and AdEAT2 co-immunization. Balb/c mice were co-immunized intramuscularly in the tibialis anterior with equivalent viral particles of Ad-HIV/Gag mixed with either Ad-GFP or Ad-EAT2 (total of 7 1×10 vps). At 6 dpi, peripheral blood mononuclear cells (PBMCs) (A) were collected from the immunized mice and stained with a PE-conjugated AMQMLKETI tetramer complex (A). At 14 dpi, mice were sacrificed and PBMCs (B) or splenocytes (C) were harvested and stained with a PE-conjugated H2-Kd-AMQMLKETI tetramer complex together with an APC-conjugated antiCD3 and FITC-conjugated anti-CD8 antibodies. The bars represent mean ± SD for six mice per group (pool of two for PBMCs) for virus injected and three mice for naïve animals. Statistical analysis was completed using One Way ANOVA with a Student-Newman-Keuls post-hoc test, 69 p<0.05 was deemed a statistically significant difference. * denotes p<0. 05, ** denotes p<0. 01Statistically different from mock injected animals. Figure 7: HIV-Gag specific cellular immune responses elicited by Ad-HIV/Gag and AdEAT2 co-immunization. 70 Following ex vivo stimulation with the immunodominant Gag peptides AMQMLKETI (QBI# 304754, for Balb/c mice) or QBI# 304796 (for C57BL/6 mice), splenocytes derived from mice co-immunized with Ad-HIV/Gag and Ad-EAT2 contained significantly (p<0. 001) increased numbers of Gag-specific, IFN-γ secreting cells (Figure 8a and b, respectively). We also observed significantly increased numbers of IL-2 secreting, HIV-Gag peptide specific (QBI# 304754) splenocytes derived from Balb/c mice co-immunized with Ad-HIV/Gag + Ad-EAT2 as compared to Ad-HIV/Gag+ Ad-GFP co-immunized mice (p<0. 01) (Figure 8a). Figure 8: HIV-Gag specific cellular immune responses elicited by Ad-HIV/Gag and AdEAT2 co-immunization. Balb/c mice (n=6) (a) or C57Bl6 mice (n=4) (b) were co-immunized intramuscularly in the tibialis anterior with equivalent viral particles of Ad-HIV/Gag mixed with 7 9 either Ad-GFP or Ad-EAT2 (total of 1×10 vps for Balb/c mice and total of 1×10 vps for C57Bl/6 mice mixed prior to injection). At 14 d. p. i., splenocytes were harvested and stimulated ex vivo with the immunodominant peptides (AMQMLKETI) for Balb/c and QBI# 304796 (EAMSQVTNSATIMMQ) for C57Bl/6. Spot forming cells (SFCs) were quantified using and ELISPOT reader. Data are presented as mean ± SD. Statistical analysis was completed using Two-Way ANOVA with a Bonferroni post-hoc test, p<0.05 was deemed a statistically significant difference. ** denotes p<0. 01, *** denotes p<0. 001. Representative data from two independent experiments are shown. 71 Figure 8: HIV-Gag specific cellular immune responses elicited by Ad-HIV/Gag and Ad-EAT2 co-immunization. 72 EAT-2 expression during HIV-Gag vaccination also facilitated a broadened induction of HIV-Gag specific T cell clones, as we observed increased numbers of Gag-specific IFN-γ and IL-2 co-secreting splenocytes responding to an expanded variety of HIV-Gag specific peptides present within the full HIV-Gag protein (i.e.: QBI# 304742, 304769, 304779, 304800, and a peptide pool (304790, 403808, and 304826) (Figure 9a and b). Figure 9: Analysis of the breadth of Gag-responses. Balb/c mice were co-immunized intramuscularly with equivalent viral particles of Ad-HIV/Gag mixed with either Ad-GFP or Ad7 EAT2 (total dose of 1×10 vps mixed prior to injection). At 14 d. p. i. , animals were terminally sacrificed, and splenocytes were harvested and stimulated ex vivo with 15mer HIV-Gag derived peptides QBI#304724, (SLYNTVATLYCVHQR), QBI#304753(GHQAAMQMLKETINE), QBI#304754 (AMQMLKETINEEAAE), QBI#304769 (PVGEIYKRWIILGLN), QBI#304779 (VDRFYKTLRAEQASQ), QBI# 304800 (GNFRNQRKTVKCFNC), or pool of three peptides (BQI# 304790, 304808, and 304826), and IFNγ (a) and IL-2 (b) ELISPOT assays were completed. Bars represent mean ± SD. Statistical analysis was completed using Two-Way ANOVA with a Bonferroni post-hoc test, p<0.05 was deemed a statistically significant difference. * denotes p<0. 05, ** denotes p<0. 01, *** denotes p<0. 001, # denotes p<0. 001Statistically different from naive animals. 73 Figure 9: Analysis of the breadth of Gag-responses. 74 In a more global analysis as to the extent of HIV-Gag peptide recognition promoted by prior expression of EAT-2, we ex vivo stimulated splenocyte preparations derived from the immunized mice with HIV-gag derived peptide pools, each pool containing 2-4 Gag specific 15mer peptides spanning the entire HIV-Gag protein sequence. We observed an increased breadth in the immune recognition of HIV-Gag by co-expression of EAT-2, as the number of HIV Gag-specific peptides that triggered cellular responses from splenocytes derived from the Ad-HIV/Gag + Ad-EAT2 co-immunized mice were significantly increased, as compared to similarly treated splenocytes derived from the Ad-HIV/Gag+ Ad-GFP control mice (Figure 10). Figure 10: “(For interpretation of the references to color in this and all other figures, the reader is referred to the electronic version of this dissertation)” Analysis of T cell epitope responses of Balb/c and C57Bl/6 mice to HIV-Gag in Ad-HIV/Gag and Ad-EAT2 coinjected mice. Balb/c (n=6) (a) or C57BL/6 (n=4) (b) mice were co-immunized with equivalent 7 viral particles of Ad-HIV/Gag mixed with either Ad-GFP or Ad-EAT2 (1×10 total vps for 9 Balb/c and 1×10 total vps for C57Bl/6 mice). At 14 dpi splenocytes were equivalently pooled and IL-2 ELISPOT analysis was carried out by stimulating individual wells ex vivo with a pool of 2-4 15mer peptides overlapped by 11, not including peptides included in Figure 4 and 5. SFCs per million splenocytes are shown. The minimal threshold response is indicated by the line above 10. 75 Figure 10: “(For interpretation of the references to color in this and all other figures, the reader is referred to the electronic version of this dissertation)” Analysis of T cell epitope responses of Balb/c and C57Bl/6 mice to HIV-Gag in Ad-HIV/Gag and Ad-EAT2 coinjected mice. 76 CD8 T cell depletion studies suggested that the majority of the T cells responding to the + antigens were CD8 (Figure 11). Figure 11: Cellular immune responses after CD8+ T cells depletion in Ad-HIV/Gag and Ad7 EAT2 co-immunized mice. At 14 dpi, splenocytes from vaccinated Balb/c mice (1×10 total vps) were equivalently pooled (N=6 mice per treatment) and CD8+ cells were depleted using 5 magnetic beads. 5×10 splenocytes were added to each well and stimulated with the immunodominant peptide AMQMLKETI. (a) A representative flow cytometric analysis before + + and after CD8 T cell depletion is shown. Spots from CD8 un-depleted cells and CD8 depleted cells were quantified using an automated ELISPOT reader (b and c). %SFC that are CD8- = + (#SFCs CD8 dep / #SFCs CD8 )*100 (d). The bars represent mean ± SD. Statistical analysis was completed using student’s t-test. 77 Figure 11: Cellular immune responses after CD8+ T cells depletion in Ad-HIV/Gag and AdEAT2 co-immunized mice. 78 Published reports have shown that the increased presence of antigen specific T cells that express several cytokines in response to antigens correlate with improved vaccine-induced protective immunity, and positively correlate with the induction of long lived memory responses (298, 299). To enumerate these "polyfunctional" T cells, we evaluated the expression of + cytokines in HIV-Gag-specific CD8 T cells generated after Ad-HIV/Gag and Ad-EAT2 co+ immunization. Six-color flow cytometry was used to enumerate the frequency of CD8 T cells producing IFNγ, TNFα, and/or IL-2 after ex vivo stimulation with HIV-Gag specific peptides. We observed statistically higher numbers of HIV-Gag-specific IFNγ-positive (p<0. 05) (Figure + 12 b and c) and IFNγ/ TNFα-double positive (p<0. 05) (Figure 12 d and e) CD8 T cells derived from Ad-HIV/Gag and Ad-EAT2 co-immunized mice as compared to mice vaccinated with the + control vaccines. When evaluating TNFα or IL-2 single positive CD8 T cells, we also observed + increased numbers of CD8 T cells that express TNFα or IL-2 derived from Ad-HIV/Gag and Ad-EAT2 co-immunized mice as compared to Ad-HIV/Gag and Ad-GFP co-immunized mice; however, with these numbers of samples these improved trends did not reach statistically significant levels (data not shown). Figure 12: Ad-HIV/Gag and Ad-EAT2 co-immunization increases the frequency of HIVGag specific CD8+ T cells. Balb/c (n=6) or C57BL/6 (n=4) mice were co-immunized with 7 equivalent viral particles of Ad-HIV/Gag mixed with either Ad-GFP or Ad-EAT2 (1×10 total 9 vps for Balb/c and 1×10 total vps for C57Bl/6 mice). At 14 dpi, the mice were sacrificed and lymphocytes were isolated from spleen. Multiparameter flow cytometry was used to determine + the total frequency of cytokine-producing CD8 T cells. (a) Representative example of the 79 + gating strategy used to define the frequency of cytokine producing CD8 T cells. Gate were set based on negative control (naïve) and placed consistently across samples. The total frequency of + CD8 T cells derived from Balb/c or C57Bl/6 mice expressing IFNγ (b and c, respectively) or IFNγ and TNFα (a and e, respectively) is shown. The bars represent mean ± SD. Statistical analysis was completed using One Way ANOVA with a Student-Newman-Keuls post-hoc test, p<0.05 was deemed a statistically significant difference. * denotes p<0. 05- Statistically different from naive animals. 80 Figure 12: Ad-HIV/Gag and Ad-EAT2 co-immunization increases the frequency of HIVGag specific CD8+ T cells. 81 + The direct measurement of in vivo functionality of CD8 T lymphocytes (CTL) to specifically kill antigen presenting target cells provides a critical assessment as to their overall + functional capacity. To evaluate the in vivo cytolytic activity of the Gag-specific CD8 T lymphocytes generated after Ad-HIV/Gag and Ad-EAT2 co-immunization, mice were coimmunized with Ad-HIV/Gag+ Ad-EAT2 or Ad-HIV/Gag+ Ad-GFP. 14 days after vaccination, the two groups of mice were then injected with carboxyfluorescein succinimidyl ester (CFSE)labeled syngeneic splenocytes pulsed with the Gag derived peptides AMQMLKETI or QBI# 304796. The elimination of the peptide-pulsed splenocytes (CFSE high ) was then examined by a flow cytometry-based CTL assay (300). Our results demonstrated that HIV-Gag specific CTL activities induced in mice co-immunized with Ad-HIV/Gag and Ad-EAT2 were significantly higher as compared to control mice (p<0. 05 for Balb/c and p<0. 01 for C57Bl/6 mice) (Figure 13 a and b). Figure 13: Increased cytolytic activity of the Gag-specific T-cell in vivo in Ad-HIV/Gag and Ad-EAT2 co-immunized mice. Balb/c (n=6) (a) or C57BL/6 (n=4) (b) mice were coimmunized with equivalent viral particles of Ad-HIV/Gag mixed with either Ad-GFP or Ad7 9 EAT2 (1×10 total vps for Balb/c and 1×10 total vps for C57Bl/6 mice). At 14 dpi, syngeneic splenocytes were pulsed with either an irrelevant peptide (NYD-pep) and stained with 1µM (CFSE Low ) (or with the HIV-Gag specific peptides (AMQ peptide for Balb/c and QBI# 304796 for C57Bl/6 mice) and labeled with 10µM (CFSE High ). Five hours after adoptive transfer into either Naïve or immunized mice, splenocytes were harvested and sorted using a LSRII flow cytometer. % CFSE positive cells were quantified using FlowJo software. % specific killing = 1- 82 ((% CFSE High / % CFSE Low ) immunized / (% CFSE High / % CFSE Low ) non-immunized). ** denotes p<0. 01, *** denotes p<0. 001- Statistically different from naive animals. Representative figure of two combined independent experiments is shown for Balb/c mice. 83 70 60 50 40 30 20 10 85 70 60 50 40 30 20 10 10 10 Figure 13: Increased cytolytic activity of the Gag-specific T-cell in vivo in Ad-HIV/Gag and Ad-EAT2 co-immunized mice. 84 To investigate the mechanisms underlying the enhanced Gag-specific adaptive immune responses generated after Ad-HIV/Gag and Ad-EAT2 co-immunization, we evaluated the expression of markers associated with APC function, in this instance analyzing bone marrow derived macrophages (BMDMs). Isolated BMDMs were initially infected with the Ad-EAT2 or Ad-GFP vectors at escalating multiplicities of infection (5,000-50,000 vps/cell). Cells were then analyzed for the surface expression of CD80 and CD86 co-stimulatory molecules by flow cytometry. Mock infected BMDMs expressed low levels of CD80 and CD86 while infection with the control Ad virus significantly induced the expression of CD80 and CD86 molecules (Figure 14). Interestingly, Ad-EAT2 infection greatly enhanced the expression of CD80 and CD86 as compared to BMDMs infected with the control Ad (Figure 14). These effects were not seen if the BMDMs were infected with a UV irradiation-inactivated Ad-EAT2 vector, confirming that EAT-2 gene expression was necessary for the effect (data not shown). Figure 14: EAT2 overexpression augments CD80 and CD86 expression by bone marrow derived macrophages. In vitro cultured murine BMDMs (500,000 cells/ well) were mock infected or infected with the Ad-EAT2 or Ad-GFP control for 72 h at the multiplicity of infection (MOI) shown. (A) Expression of GFP on BMDMs following infection with escalating doses of Ad-GFP at 72 h. (B) Expression of CD80 on BMDMs 72 h postinfection with escalating doses of Ad-EAT2 (black) or Ad-GFP (gray). (C) CD86 expression in BMDMs infected with various doses of Ad-EAT2 or Ad-GFP at 72 h postinfection. Data are representative of three independent experiments with similar results. Samples were plated in triplicate and are expressed as mean ± SD (* P<0.05, ** P<0.01, *** P< 0.001 statistically different from uninfected cells). 85 Figure 14: EAT2 overexpression augments CD80 and CD86 expression by bone marrow derived macrophages. 86 RAW264.7 cells were also infected with either the Ad-EAT2 or Ad control vector. Similar to the results we obtained in BMDMs, Ad infection resulted in significant increases in the expression of CD40, CD80, CD86, and MHC-II molecules (Figure 15). Importantly, AdEAT-2 infection resulted in significantly higher levels of CD40, CD80, CD86, and MHC-II molecules as compared to cells infected with the Ad control virus (Figure 15). Figure 15: Increased expression of CD40, CD80, CD86, and MHC-II in Ad-EAT2 infected RAW264.7 cells. RAW264.7 cells (500,000 cells/ well) were mock infected or infected with 20,000 vector particles/ cell of Ad-EAT2 (black or Ad-Null (gray). Seventy-two hours later, cells were stained with antibodies specific for CD40, CD80, CD86, and MHC class II, and analyzed on an LSR-II flow cytometer. Data are representative of four independent experiments with similar results. Samples were plated in triplicate and are expressed as mean ± SD (*** denotes P< 0.001 statistically different from uninfected cells). 87 Figure 15: Increased expression of CD40, CD80, CD86, and MHC-II in Ad-EAT2 infected RAW264.7 cells. 88 2.3. Discussion The SLAM family of receptors modulate multiple innate and adaptive immune responses through their intracellular signaling adaptors, SAP, EAT-2, and ERT (141, 290, 301). This report provides evidence supporting an important new strategy to improve the efficacy of vaccines in general, and that of Ad based vaccines specifically, by expression of SLAM system derived adaptors simultaneous with antigen specific vaccinations. More specifically, in this study we confirmed these notions by utilizing an Ad vaccine genetically engineered to express the SLAM adaptor molecule EAT-2 along with the HIV derived antigen Gag. The inclusion of the AdEAT2 vector in Ad-HIV-gag vaccine cocktails significantly impacted upon the innate immune responses induced by the vaccine cocktail, and more importantly specifically improved multiple, antigen specific adaptive (cellular) immune responses to the target antigen present in the vaccine cocktail, in this instance the HIV Gag protein. There are a number of mechanisms as to how expression of EAT-2 might facilitate induction of antigen specific immune responses in vivo. NK cells represent a subset of innate immune cells that have been shown to play an important role in bridging innate and adaptive immune responses, by influencing DC function (302), providing signals for augmenting Th1 immune responses (77, 78, 303), and inducing tumor-specific CTLs (304). In addition, it has been shown recently that NK cell-mediated cytotoxicity of antigen-expressing target cells induces robust antigen-specific adaptive immune responses (80). EAT-2 has been shown to be indispensable in activating NK cell mediated cytotoxicity, by acting as a downstream adaptor protein facilitating signaling from the SLAM family receptor CRACC (in mice) (151, 305) or NTB-A (in humans) (306). Ad mediated transduction of the EAT-2 gene enhanced NK cell activation (especially prolonged in those NK cells found in the circulation), activations that may 89 facilitate the activation and/or maturation of DCs thereby biasing the HIV-Gag specific immune profile towards a Th1 response (78). Interestingly, we noted that Ads induced significantly elevated levels of IFN-γ in NK cells regardless of EAT-2 overexpression. These results suggest that EAT-2 overexpression enhances immune responses to the co-injected antigen in a mechanism that does not involve induction of higher levels of IFN-γ producing NK cells. For example, in addition to NK cells, we also observed increased activation of NKT cells after Ad mediated transduction of the EAT-2 gene. Several reports have shown that enhancing the activation of NKT cells can also positively influence the initial activation of DCs and/or NK cells, thereby increasing DC-dependent adaptive (cellular) immune responses (85-89). Our data suggests that harnessing this potential capability of NKT cells (in this instance via expression of EAT-2) may be an important goal of “next generation" vaccines. Previous reports have shown that SLAM derived signaling can also increase the antigen presentation capabilities of APCs (291, 292). We note that EAT-2 is the only SLAM adaptor molecule currently known to be expressed in APCs (290). These previous observations, suggest that Ad mediated transduction of EAT-2 into DCs may have directly facilitated the induction of antigen specific adaptive immune responses observed in this study. We confirmed that EAT-2 over-expression within several different types of APC triggers the induction of CD40, CD80, CD86, and MHC class-II, all of which are critical co-stimulatory molecules that augment subsequent APC activation of T cells. While it is known that Ads can transduce APCs and DCs in vivo, other cell types may also be being transduced by the Ad vector expressing EAT-2, and also be impacting upon our in vivo results. It is conceivable that EAT-2 overexpression also facilitated SLAM - independent functions in Ad-EAT2 transduced immune cells. SAP family adaptors can also interact, by way of their SH-2 domain, with other classes of receptors 90 - expressed in immune cells including CD22 (expressed in both CD8α DCs and B cells) and FcγRIIB (307, 308). Future studies will be required to delineate which aspects of the augmented EAT-2 dependent immune responses may be mediated by these or other receptors. Various studies in mouse models and non-human primates suggest that improving the breadth of the antigen specific cellular immune responses elicited by a vaccine is positively correlated with an improved ability of the vaccine to induce protective immunity for example after pathogen challenge of vaccinated subjects (309, 310). Our results demonstrate that vaccine cocktails possessing the ability to express EAT-2 along with a target antigen increased the breadth of the cellular immune responses to the antigen, a result that correlated with a significantly improved induction of antigen specific cytolytic T cell activity in vivo. We confirmed these results in both C57BL/6 and Balb/c mice, (two mouse strains that can bias adaptive immune responses to a Th1 or Th2 response, respectively) indicating that the "adjuvant" effect of EAT-2 is not specifically limited by immuno-genetic background differences of the host animal, at least in this species. In conclusion, our findings suggest that enhancing SLAM signaling by expressing EAT-2 during antigen vaccination can serve to improve the ability of a vaccine to stimulate the innate immune system, and subsequently induce improved, antigen specific adaptive immune responses. Acknowledgment: We wish to thank Michigan State University Laboratory Animal support facilities for their assistance in the humane care and maintenance of the animals utilized in this work. We specifically thank, Dr. Sungjin Kim for his generous advice and suggestions. A.A. was supported by the MSU Foundation, as well the Osteopathic Heritage Foundation. YAA was 91 supported by the King Abdullah bin Abdulaziz Scholarship, Ministry of Higher Education, Kingdom of Saudi Arabia. 92 Chapter III Vaccine platforms combining Circumsporozoite protein and potent immune modulators, rEA or EAT-2, paradoxically result in opposing immune responses This chapter is the edited version of a research article that was published in PLoS ONE Journal, Volume 6, Issue 8 (e24147), August 30, 2011. Authors: Aldhamen Y.A.*, Schuldt N.J.*, Appledorn D.M., Seregin S.S., Kousa Y., Godbehere S., and Amalfitano. *these authors contributed equally to this work. Authors’ contribution: Aldhamen Y.A.*: Constructed the rAd5-EAT2 vaccine vectors, performed all the rAd5-CSP+ rAd5-EAT2 co-injection experiments [except the in vivo CTL assays], performed and analyzed CSP-tetramer and ICS staining for rAd5-CSP+ rAd5-GFP/rEA, developed the protocol of in vivo CTL assay and guide in performing the in vivo CTL testing for rAd5-CSP+ rAd5-EAT2 coinjection experiments, and gave some advice during manuscript writing. Schuldt N.J. *: Performed all the rAd5-CSP+ rAd5-GFP/rEA co-injection experiments, preformed in vivo CTL experiments for rAd5-CSP+ rAd5-EAT2 co-injection experiments, performed all the ELISA analysis, and wrote the manuscript. All other co-authors: Helped in performing experiment and participated in discussion during manuscript writing. 93 3.1. Introduction In this chapter, I will provide a brief background about malaria and Plasmodium parasites, the immune responses to malaria parasite infections, describe the current state of the malaria vaccine field and outline strategies for the development of an effective malaria vaccine, and finally introduce a novel strategy for developing a pre-erythrocytic-stage malaria vaccine by utilizing adenoviral vectors that express the Plasmodium falciparum circumsporozoite protein and the innate immune modulators rEA or EAT-2 proteins. Malaria is an infectious disease that continues to devastate populations world-wide, causing nearly 1 million deaths annually, and morbidity that overwhelms the medical capabilities of developing countries. Malaria is caused by several species of the Plasmodia parasite that have very complex life cycles involving a female Anopheles mosquito and a mammalian host. The plasmodium parasite life cycle is a very complex multi-stage process including both obligate intracellular asexual growth within mammalian hepatocytes (the pre-erythrocytic stages) and erythrocytes (the blood or erythrocytic-stages) (311). Sexual differentiation, including the fusion of gametocytes and parasite propagation, occurs in the mosquito vector (311). Five species of Plasmodium are infectious for human: P. falciparum, P. vivax, P. malariae, P. ovale, and P. knowlesi. Most cases of Malaria are caused by an infection with the protozoan parasite P. falciparum. Plasmodium falciparum infections are responsible for about 80% of all malaria cases and around 90% of all malaria deaths, therefore it has been the focus of most research (312). However, recent studies in South East Asia have also shown that 25% of patients with severe malaria also have P. vivax infection (313). 94 3.1.1 Immune responses to Plasmodium parasites Similar to other microbial infections, infections by Plasmodium parasites have been associated with enhanced activation of the innate immune system followed by adaptive immune responses involving both B- and T-cells (314). Plasmodium parasite infections result in activation of several innate immune cells including NK cells, NKT cells, DCs, Kuppfer cells, γδ T cells, and macrophages (315). Several pro-inflammatory cytokines, such as TNFα, IL-1, IL-6, IFNα and IFNγ, have been detected in responses to Plasmodium infection (316), thus stimulating and regulate the adaptive immune responses. In addition, soluble products from Plasmodium parasites have been shown to induce IFNα production from pDCs and to increase expression of CD86 and CCR7 on pDCs in vitro (316). The induction of these pro-inflammatory cytokines has been shown to require functional TLR (TLR2 and TLR9) and Myd88 signaling pathways (316, 317). + + CD8 T cells and CD4 T cells have been shown to be activated by parasitic infection (318). Studies in murine models and humans have demonstrated that T cells were important for pre-erythrocytic (sporozoite-liver) stage immunity (319). In particular, the sporozoite’s major + surface protein, the Circumsporozoite (CS) protein, was shown to be a target for CD8 T cells (320). T cell depletion studies prior to WT-sporozoite challenge in Balb/c mice have identified + CD8 T cells as the primary cytotoxic cells in radiated-sporozoite induced immunity (321). In + addition, it has been shown that after an infectious mosquito bite, CS Protein-specific CD8 T cells, primed by skin-draining lymph nodes activated DCs, migrate to the liver and eliminate + parasite infected hepatocytes (322). Moreover, studies have also revealed that CD8 T cellderived IFNγ, but not the lytic factors (perforin, Granzyme B, or Fas), are the main effector 95 + molecule that mediates protection following WT-sporozoite challenge (323). CD4 T cells have also been shown to play a critical role during parasite infection in murine malaria models. For + example, immunization of β2-microglobulin deficient mice (which fail to induce CD8 T cell responses) with either P. berghei or P yoelii radiated sporozoites, rendered these mice completely protected from subsequent challenge (324). Similarly, studies utilizing genetically + + attenuated whole parasite (GAPs) immunization strategies have also shown that CD4 , CD8 T cells, and IFNγ are each key mediators of protective immunity (325). Gamma-delta T cells have also been demonstrated to contribute to protection in P. yoelii radiation sporozoite-immunized mice lacking conventional αβ T cells (326). In addition, regulatory T cells have also been shown to play a role during plasmodium infection. Treg cells have been shown to associate with increased IL-10 and TGFβ secretion, diminished pro-inflammatory cytokine production, and decreased antigen-specific immune responses (327). The role of B cell responses to malaria parasites has also been studied. During natural infection, antibodies against both the merozoite surface proteins (MSPs) and erythrocyte membrane-associated antigens (EMPs) have been detected. These antibodies were found to have growth inhibitory properties in vitro including, inhibiting invasion of new erythrocytes, blocking the sequestration of infected erythrocytes to endothelial cells, and promoting opsonization, thus enhancing phagocytic activity of monocytes and macrophages (328). However, studies in both humans and mice have shown that memory B cell responses are poorly induced or are short-lived as a result of natural infection. Despite these immune responses, malaria parasites have developed a variety of mechanisms that allow them to evade immunity and persist in the host. Several studies have 96 shown that enhancing apoptosis of T cells and other effector cells (329), interfering with presentation and processing (330), mutating the sequence of epitopes critical for T- or B-cell recognition (331), and induction of T and B cell exhaustion (332), are all mechanisms that have been described for immune evasion by Plasmodium parasites. 3.1.2 Malaria vaccine development It is known that prophylactic vaccination of subjects with irradiated sporozoites can result in 94% protection against subsequent malaria infection, a result confirming that induction of protective immunity to malaria antigens can be achieved, if the vaccine utilized is potent enough and expresses the correct antigenic targets (314, 333). Unfortunately, the large scale production of irradiated sporozoites for this purpose has not been feasible, largely due to the difficulties associated with cGMP production of this class of vaccine. Significant efforts have been undertaken to create malaria specific subunit vaccines. Malaria subunit vaccines that target blood-stage malaria infection with recombinant P. falciparum erythrocyte membrane protein-1 (PfEMP-1) or merozoite surface proteins have failed to achieve protection against malaria in humans, despite production of strong antibody responses (334). Some of the most successful malaria vaccine studies that target the liver-stage, to date, have attempted to induce adaptive immune responses to the P. falciparum CS protein. CS protein is an ~58 kDa surface protein composed of a middle repeat region consisting of multiple NANP repeats that are flanked by a C-terminus containing a thrombospondin-like type I repeat region (TSR) and an N terminal region that assists with liver cell attachment (Figure 1) (335). Figure 16: CS protein sequence. The CS protein sequence utilized for constructing the Ad-CSP vaccine was designed based on several known CS protein sequences. The NYDNAGTNL 97 peptide’s location is underlined in the sequence. Bold font within the sequence indicates the repeat region of CS protein. The location of the Thrombospondin-like Type 1 repeat region (TSR domain) is indicated by gray font. 98 Figure 16: CS protein sequence. 99 CS protein is expressed on the surface of sporozoites and is also expressed in the plasma membrane and cytoplasm of infected hepatocytes during early liver infection (336). Induction of potent cellular immune responses to CS protein by a prophylactic malaria vaccine could potentially eradicate both sporozoites and infected hepatocytes, potentially stopping the infection before clinical symptoms occur. + Multiple studies have demonstrated the importance of CD8 T cell responses in combating murine malaria infections (337-340). An oral salmonella vaccine expressing murine malaria derived CS protein was capable of protecting antibody deficient animals (339). In + addition, passive transfer of CD8 T cells that recognize a specific murine malaria CS protein antigen resulted in 100% survival upon sporozoite challenge (338). Ads expressing murine malaria derived CS protein have been shown to be capable of providing cytotoxic T cell mediated inhibition of parasite liver stage development up to 93% (340). One of the most successful subunit malaria vaccines to date contain a hepatitis B viral surface protein (HBsAg) CS fusion protein (referred to as RTS,S). Initial formulations of the RTS,S based vaccines demonstrated little protection. Subsequent trials combining several novel “AS” series adjuvants, (the latter consisting of different preparations of monophosphoryl lipid A (MPL) and a plant extract known as QS21), with the HBsAg-CSP fusion protein (referred to as RTS,S/ASO1B) improved induction of CS specific responses, but this level of protection was still limited, suggesting that a more robust CS protein specific immune response may be required to achieve improved protection rates (341-347). Recombinant adenoviruses (rAd) can be utilized to induce potent adaptive immune responses to antigens that they are genetically engineered to express. For example, rAd serotypes 5 and 35 expressing CS protein (Ad5.CS and Ad35.CS, respectively) are capable of inducing CS 100 protein specific T and B cells in mice similar to the levels induced by the RTS,S/ASO1B adjuvanted vaccine (348, 349). These levels paralleled results achieved in mice treated with a rAd35 vaccine expressing the P. Yoelli derived CS protein, a vaccine that was also found to be capable of providing up to a 92-94% inhibition of liver infection when vaccinated animals were challenged intravenously (IV) with viable P. yoelli sporozoites (350). Combining rAd35CS and rAd5CS CS protein expressing vaccines in a heterologous prime-boost regimen in rhesus monkeys can induce a more potent T and B cell response than use of either vaccine alone (351). Heterologous prime-boost vaccination regimens utilizing Ad35.CS protein and RTS,S/ASO1B have also been analyzed and show significant improvement over either vaccine platform alone (352). However, the utilization of alternative serotype rAds or chimp derived rAds as a vaccine platform may be dangerous, as our studies and those of others have confirmed the increased innate toxicity of non-Ad5 rAds (353). Therefore, improving the capability of rAd5 vaccines to induce more potent antigen specific adaptive immune responses is a high priority in the drive to find an efficacious malaria vaccine. In this study we sought to improve CS protein specific cell mediated immune (CMI) responses induced by CS protein-expressing rAd5s by co-expression of innate immune response modulating proteins by the vaccine platform. The innate immune system plays an integral role in augmenting and/or shaping the induction of antigen specific adaptive immune responses (2). A group of cellular receptors that recognize a variety of pathogen derived antigens, known as the toll-like receptors (TLRs), play a crucial role in identifying PAMPs, and then augmenting adaptive responses to those PAMPs. We have previously confirmed that rAds ability to induce innate and adaptive responses are dependent upon several TLR’s, and that many of these responses are primarily dependent upon MyD88 functionality (179, 184). We have also recently demonstrated that when rAd5 vaccines 101 engineered to express a novel Eimeria tenella derived TLR agonist, rEA, are co-administered with rAd5 vaccines expressing a target antigen there was significant improvement in the ability of the vaccine to induce antigen specific cellular immune responses (133, 354, 355). Similarly, we have recently confirmed that co-expression of adaptor proteins derived from SLAM receptors signaling pathways can also augment induction of beneficial immune responses to rAd expressed antigens (191, 356). In the latter instance we utilized the SLAM family of receptors adaptor protein (EAT-2), an adaptor protein known to mediate SLAM receptor signaling in innate immune cells (357, 358). In this study, we determined what the impact of modulation of innate immune responses during CS protein presentation would have upon induction of subsequent CS protein specific immune responses in vivo. Unexpectedly, use of a TLR agonist uncovered a potent immunosuppressive activity inherent to the combined use of rEA and CS protein, an activity that mitigated induction of any CS protein specific adaptive immune responses. Fortunately, expression of the SLAM receptors adaptor protein EAT-2 overcame and enlightened possible mechanisms underlying the paradoxical CS protein immunosuppressive activity we uncovered when stimulating TLR pathways. 102 3.2 Results CS protein expressed from rAd5 based vaccines can induce CS protein specific B and T cell responses. A rAd5 based vaccine expressing a codon optimized form of the CS protein (AdCSP) was constructed as shown in figure 17. Figure 17: Ad-CSP construction. Recombinant Ad-CSP was constructed by creating a codon optimized CS protein sequence flanked by NheI sites in a pGA4 plasmid. The sequence was excised with the Nhe1 and cloned into a pShuttle containing a CMV expression cassette. The resulting plasmid was linearized with PmeI and recombined with pAdeasyI Ad5 vector in BJ 5183 cells. pAd-CSP was then purified and linearized with PacI enzyme and transfected into HEK 293 cells from which Ad-CSP was purified using cesium gradients. 103 Figure 17: Ad-CSP construction. 104 A dose response study was initially performed to assess at what dose optimal CS specific B and T cell responses could be detected. BALB/cJ mice were intra-muscularly (IM) injected 7 9 with varying doses of Ad-CSP ranging from 1×10 to 1×10 virus particles (vps) per mouse. At 14 days post injection (dpi), splenocytes derived from the vaccinated mice were harvested and exposed to an immunodominant CS protein derived peptide (NYDNAGTNL). Significantly increased numbers of IFNγ secreting splenocytes were noted in Ad-CSP vaccinated mice treated 7 9 8 with 5.0×10 to 1.0×10 vps, with peak numbers achieved at a dose of 1.0×10 vps/mouse. Higher Ad-CSP doses resulted in a trend of decreasing, though not significantly less, numbers of spot forming cells (SFCs) (Figure 18A). Interestingly this phenomenon has also been observed by other groups; however, an explanation for this phenomenon has yet to be provided (359, 360). These finding were further supported in individual splenocytes derived from the vaccinated + mice, where CD8 T cell IFNγ, TNFα, and IL-2 levels were measured by intracellular staining 8 (ICS) using flow cytometry. IFNγ and TNFα production peaked at the 5.0 ×10 vps/mouse with 9 similar decreasing trend occurring at 1.0 ×10 vps/mouse. IL-2 producing cells were much lower 7 in percentage, with the greatest numbers being observed as the 5.0×10 vps/mouse dose (Figure 18B). We will further discuss the importance of these findings in the discussion section. To determine if Ad-CSP is also capable of stimulating B cell responses specific to the CS protein, plasma was collected from the vaccinated mice and assayed by an IgG CS protein specific ELISA at 14 dpi. Significant increases in CS specific IgG were detected in all mice 7 treated with Ad-CSP with a peak response occurring at 5.0×10 vps/mouse, demonstrating Ad- 105 CSP is capable of stimulating a B cell response against CS protein even at the lowest dose used in the study (Figure 18C). Figure 18: Ad-CSP Stimulates CS protein specific T and B cell responses. CS protein specific immune responses increase in an Ad-CSP dose dependent manner. BALB/cJ mice (n=3) 7 9 were injected IM with Ad-CSP ranging from 1x10 to 1x10 vps/mouse, increasing by half logs. 14 days post injection splenocytes and plasma were collected. (A) ELISpot assays were performed to quantify IFNγ secreting cells from splenocytes stimulated with CS protein peptide, NYDNAGTNL, ex vivo. (B) IFNγ, TNFα, and IL-2 expression by splenocyte derived CD3 + + CD8 T cells was analyzed by flow cytometry following ex vivo stimulation with NYDNAGTNL. (C) Total IgG against CS protein was assessed by ELISA. The bars represent mean ± SD. Statistical analysis was completed using One Way ANOVA with a StudentNewman-Keuls post-hoc test, *,**,*** denotes significance over naïve, p<0.05, p<0.01, p<0.001. 106 Figure 18: Ad-CSP Stimulates CS protein specific T and B cell responses. 107 Previous experiments confirm that expressing the TLR agonist rEA from an Ad vector stimulates innate immune responses during Ad-mediated vaccination, responses that positively correlated with improved induction of antigen specific adaptive immune responses against several antigens, such as the HIV antigen, Gag (133). In this study, we sought to utilize rEA to improve induction of CS protein specific immune responses. We first confirmed that expression of rEA along with CS protein facilitated induction of pro-inflammatory innate immune responses, responses we had noted in our previous studies of rEA. Plasma cytokine levels at 6 10 hours post injection (hpi) in mice co-injected intravenously (IV) with either 3.75×10 10 Ad-CSP and 3.75×10 vps of vps Ad-GFP/rEA were compared to responses measured after identical co-injections utilizing an Ad-GFP expressing vector (that does not express rEA) as a control (Figure 17) . We observed significantly higher levels of IL-6, IL-12(p40), G-CSF, MCP-1, MIP1β, RANTES, KC, and TNFα in mice treated with Ad-CSP +Ad-GFP/rEA as compared to control virus treated animals, as well as, mock infected animals (Figure 19). Figure 19: TLR agonist, rEA, induced innate cytokines 6 hours post injection. Co-injection of Ad-GFP/rEA and Ad-CSP stimulated robust expression of innate cytokines and chemokines 10 as compared to the control vaccine. BALB/cJ mice were injected IV with either 3.75×10 10 vps/mouse of Ad-CSP +Ad-GFP or 3.75×10 vps/mouse Ad-GFP/rEA +Ad-CSP. Plasma was collected at 6 hours post injection. Plasma cytokine/chemokine levels were measured with a mouse multiplexed bead array based quantitative system. The bars represent mean ± SD. Statistical analysis was completed using One Way ANOVA with a Student-Newman-Keuls posthoc test, *,** denotes significance between treatments, p<0.05, p<0.01. 108 Figure 19: TLR agonist, rEA, induced innate cytokines 6 hours post injection. 109 To assess the impact that these early increases in cytokine and chemokine responses had 7 on cell mediated immune (CMI) responses to CS protein we IM co-injected 5×10 vps/mouse of 7 Ad-CSP and 5×10 vps/mouse of Ad-GFP/rEA and compared the induction of CS specific 7 adaptive immune responses to those noted in our control mice receiving 5×10 vps/mouse of Ad7 CSP and 5×10 vps/mouse of Ad-GFP IM, or mock infected mice. Splenocytes derived from mock vaccinated mice did not show the presence of CS protein specific CMI responses while Ad-CSP +Ad-GFP confirmed induction of CS protein specific CMI responses using ELISpot analysis (p<0.05) (Figure 20A). However, despite the rEA enhanced activation of the innate immune responses noted in Figure 19, ELISpot analysis of splenocytes derived from Ad-CSP+ Ad-GFP/rEA vaccinated animals confirmed a profound lack of induction of average CS protein specific CMI responses, responses that were essentially identical to CS responses measured in naïve mice (p>0.05) (Figure 20A). Figure 20: Immuno-modulating proteins conversely affect IFNγ secreting splenocytes. Co-vaccination with Ad-CSP and Ad-EAT2 dramatically increases IFNγ secreting splenocytes in response to stimulation with CS protein epitope, NYDNAGTNL. BALB/cJ mice were injected 7 7 7 IM with either 5×10 vps/mouse of Ad-CSP and 5×10 vps/mouse Ad-GFP or 5×10 vps/mouse 7 of Ad-CSP and 5×10 vps/mouse of either (A) Ad-GFP/rEA (n=5) or (B) Ad-EAT2 (n=6). Splenocytes were collected 14 days post co-injection. ELISpot were performed on the splenocytes of these mice stimulated with NYDNAGTNL peptide to assess the amount of IFNγ secreting cells. The bars represent mean ± SD. Statistical analysis was completed using One Way 110 ANOVA with a Student-Newman-Keuls post-hoc test,* Denotes significance over naïve animals, p<0.05. Representative figures of two independent experiments. Figure 20: Immuno-modulating proteins conversely affect IFNγ secreting splenocytes. 111 Previously we have not observed an ablation of CMI responses when CS protein was coadministered with Ads expressing other antigens at these low doses, further suggesting that this effect may be specific to simultaneous TLR stimulation (Figure 21). Figure 21: CS protein expression does not interfere with antigen specific immune responses against other transgenes at low doses. Co-vaccination with Ad-Gag +Ad-CSP did not result in 5 decreased gag specific immune responses. BALB/cJ mice were injected with 5×10 vp/mouse of 7 5 7 Ad-Gag and 5x10 vp/mouse of Ad-CSP or 5×10 vp/mouse of Ad-gag and 5×10 vp/mouse of Ad-GFP. Splenocytes were collected 14 dpi and assayed by ELISpot for CS protein peptide (NYDNAGTNL) specific IFNγ secretion (A) or gag peptide (AMQMLKETI) specific IFNγ secretion (B). The bars represent mean ± SD. Statistical analysis for Supplemental Figure 3A included other peptides tested from the peptide library that are not displayed in the graph. Two Way ANOVA with Student-Newman-Keuls post-hoc test (A) or One Way ANOVA with a Student-Newman-Keuls post-hoc test (B) were utilized for statistical analysis. **, *** denotes significance between treatments, p<0.01, p<0.001. 112 Figure 21: CS protein expression does not interfere with antigen specific immune responses against other transgenes at low doses. 113 Despite there being no significant differences between CS protein responses in Ad-CSP +Ad-GFP/rEA treated animals and naïve animals, we did note that in one Ad-CSP +AdGFP/rEA animal there was some evidence of an elevated CS protein specific response, independently verifying that this group did in fact receive viable Ad-CSP vector (Figure 20A). Based upon the loss of CS protein responsiveness after utilizing TLR-mediated augmentation along with CS protein antigen vaccination we hypothesized that the CS protein may have an ability to mitigate induction of beneficial innate immune responses in the context of excessive, TLR pathway mediated activation as the ablated immune responses were only observed after Ad6 GFP/rEA doses exceeded 5×10 vp/mouse (Figure 22). 7 Figure 22: Ad-GFP/rEA combined with 5x10 vp/mouse of Ad-CSP begins to display a 6 diminished CS protein specific CMI response after a dose of 5×10 vp/mouse. Only after the 6 dose of Ad-GFP/rEA exceeds 5×10 vp/mouse do we observe a diminished CS specific CMI 6 response when combined with 5x10 vp/mouse of Ad-CSP. BALB/cJ mice were injected with 6 8 7 doses ranging from 5×10 to 5x10 vp/mouse of Ad-GFP/rEA combined with 5x10 vp/mouse of Ad-CSP. Splenocytes were collected 14 dpi and were analyzed by flow cytometry for + + + NYDNAGTNL tetramer CD3 and CD8 cells (A) or ELISpot for CS protein specific IFNγ secretion (B). Statistical analysis was completed using One Way ANOVA with a StudentNewman-Keuls post-hoc test, *** denotes significance between treatments, p<0.01, p<0.001. 114 7 Figure 22: Ad-GFP/rEA combined with 5x10 vp/mouse of Ad-CSP begins to display a 6 diminished CS protein specific CMI response after a dose of 5×10 vp/mouse. 115 To attempt to test this hypothesis, we made use of a recently described, alternative method for augmenting induction of antigen specific adaptive immune responses, utilizing Ad mediated co-expression of a SLAM receptor signaling pathway adaptor, EAT-2, along with a 7 targeted antigen (191, 356). To accomplish this we co-injected 5×10 vps/mouse of Ad-CSP and 7 5×10 vps/mouse of Ad-EAT2, and compared the induction of CS specific adaptive immune 7 7 responses to those noted in the control mice receiving 5×10 vps/mouse of Ad-CSP and 5×10 vps/mouse of Ad-GFP IM, as well as mock vaccinated mice. Again, splenocytes were collected at 14 dpi and stimulated with the CS derived peptide NYDNAGTNL ex vivo. In dramatic contrast to our previous results utilizing the Ad-GFP/rEA and Ad-CSP vaccination strategy, splenocytes from mice co-treated with Ad-CSP and Ad-EAT2 had significantly more IFNγ secreting cells than splenocytes from both mock injected mice as well as mice co-treated with the control vaccine (p<0.05) (Figure 20B). Given these results, we sought to further characterize the EAT-2 dependent improvement in CS specific immune responses by flow cytometry. Peripheral blood mononuclear cells (PBMC) derived from the vaccinated animals were stained with CD3 and CD8 fluorescent antibodies, as well as a NYDNAGTNL peptide loaded tetramer. AdCSP+Ad-EAT2 treated mice had significantly higher percentages of CS protein specific tetramer positive CD8+cells present in their PBMCs than the percentage noted in the Ad-CSP +Ad-GFP + + control group (p<0.001) (Figure 23A). CD3 CD8 splenocytes were additionally analyzed for + + IFNγ and perforin by ICS using flow cytometry. The percent of CD3 CD8 cells that secreted IFNγ was significantly higher in Ad-CSP+Ad-EAT2 treated mice as compared to Ad-CSP +AdGFP treated control (p<0.05) (Figure 23B). The percent of CS protein peptide specific CD3 116 + + + CD8 perforin cells also tended to be higher in animals given the Ad-EAT2+Ad-CSP vaccination cocktails however this did not reach statistical significance (Figure 23C). Figure 23: Co-expression of CS protein and EAT-2 stimulates more potent CS protein specific CMI responses. Co-vaccination with Ad-CSP and Ad-EAT2 resulted in increased + + NYDNAGTNL tetramer positive CD8 T cells as well as improved IFNγ secretion from CD8 T 7 cells. BALB/cJ mice (n=6) were co-injected IM with 5x10 vps/mouse of Ad-CSP and 5x10 7 7 7 vps/mouse of Ad-EAT2 or 5x10 vps/mouse of Ad-CSP and 5x10 vps/mouse of Ad-GFP. (A) Peripheral Blood Mononuclear Cells (PBMCs) were stained with CD8-Alexa Flour700, CD3APC-Cy7, and CSP (NYD)-Tetramer. (B-C) Intracellular staining was performed on splenocytes after stimulation with NYDNAGTNL peptide. Cells were stained with CD8-Alexa Flour700, CD3-APC-Cy7, ViViD, IFNg-APC, and Perforin-PE antibodies. The bars represent mean ± SD. Statistical analysis was completed using One Way ANOVA with a Student-Newman-Keuls posthoc test, *, **, *** denotes significance over naïve animals, p<0.05, p<0. 01, p<0.001 117 Figure 23: Co-expression of CS protein and EAT-2 stimulates more potent CS protein specific CMI responses. 118 To confirm that the differences in the responses observed are not a result of GFP antigens competing with CS protein antigens, but are in fact a direct result of the expression of EAT-2 we injected mice with Ad-CSP +Ad-GFP or Ad-CSP + an empty Ad vector (Ad-Null). We observed no differences between the treatments, indicating GFP does not interfere with induction of CS protein specific CMI responses (Figure 24). Figure 24: Expression of GFP does not interfere with CS protein specific CMI responses. Co-injection of Ad-GFP does not interfere with Ad-CSP initiated CS protein specific CMI 7 responses. BALB/cJ mice were co-injected with 5×10 vp/mouse of Ad-GFP and 5x10 7 7 7 vp/mouse of Ad-CSP or 5×10 vp/mouse of Ad-Null and 5x10 vp/mouse of Ad-CSP. + + + Splenocytes were collected 14 dpi and cells were measured for NYDNAGTNL tet , CD3 CD8 + + + T-cells. Both treatments had a higher percentage of CS protein specific tet , CD3 CD8 T-cells than Naïve with no difference observed between Ad-CSP + Ad-Null and Ad-CSP +Ad-GFP. Statistical analysis was completed using One Way ANOVA with a Student-Newman-Keuls posthoc test, *** denotes significance between treatments, p<0.01, p<0.001 119 Figure 24: Expression of GFP does not interfere with CS protein specific CMI responses. 120 Increased breadth of CMI responses to a pathogen derived protein has been shown to be beneficial relative to eventual protection against actual pathogen challenge (262, 309, 361). To detect CMI responses against other peptides present within the CS protein (and therefore to gauge the breadth of response against the whole CS protein) we generated a CS protein specific peptide library. This library consists of 15 mer peptides that overlap each other by 5 amino acids and spans the non-repeating regions of the full length CS protein. At 14 dpi, pooled splenocytes derived from the control or experimental groups of vaccinated animals were stimulated ex vivo with one 15mer peptide per well. Mice co-vaccinated with Ad-CSP and Ad-GFP/rEA had an overall lower breadth of response as is evident by the number of wells with more than 15 spots (Figure 25A). In contrast to the response seen in rEA treated animals, animals co-vaccinated with Ad-CSP and Ad-EAT2 demonstrated a dramatic increase in breadth of response to CS derived peptides when similarly analyzed (Figure 25B). Figure 25: Co-expression of CS protein and EAT-2 increases the breadth of response against CS protein. Increased breadth of response against CS protein epitopes was observed in mice co-vaccinated with Ad-CSP and Ad-EAT2 as compared to the control vaccine. Splenocytes from groups of five BALB/cJ mice were collected and pooled together from 14 days post injection with either innate modulating treatments or control. ELISpots were performed to measure IFNγ secreting cells when stimulated with a CS protein peptide library made up of 52 15mers that overlap by 5 aa on either side. Wells that contained more than 15 spots were counted and compared between treatment groups (inset). (A) Mice were co-injected IM with 5x107 7 7 vps/mouse of Ad-CSP and 5x10 vps/mouse of Ad-GFP/rEA or 5×10 vps/mouse of Ad-CSP 7 7 and 5×10 vps/mouse of Ad-GFP. (B) Mice were co-injected IM with 5x10 vps/mouse of Ad7 7 7 CSP and 5×10 vps/mouse of Ad-EAT2 or 5x10 vps/mouse of Ad-CSP and 5×10 vps/mouse 121 of Ad-GFP. As a negative control, naïve splenocytes were also tested against paired peptides from the peptide pool, with an average background of spots per paired peptides being only 2.2 spots. 122 Figure 25: Co-expression of CS protein and EAT-2 increases the breadth of response against CS protein. 123 To better assess the functional consequence of the improved CS specific CMI responses noted by expression of EAT-2, we stimulated splenocytes from naïve mice, mice vaccinated with the control vaccine, and mice vaccinated with Ad-CSP +Ad-EAT2 with NYDNAGTNL ex vivo, + + then analyzed them by flow cytometry for CD3 , CD8 T cells that were also positive for a degranulation marker, CD107a. Both control treated and Ad-CSP +Ad-EAT2 treated mice + + demonstrated a significantly higher number of CD8 , CD107a T cells than those quantified in + naïve mice, indicating increased ability of CD8 T cells to express granules when stimulated with a CS protein epitope (Figure 26). However, the assay was not sensitive enough to measure a difference between the control vaccinated mice and Ad-CSP+Ad-EAT2 vaccinated mice. Figure 26: Improved degranulation of CD8+ T cells in mice co-vaccinated with Ad-CSP and Ad-EAT2. Degranulation marker, CD107a, expression in CD8+ T cell from mice covaccinated with Ad-CSP+Ad-EAT2 or Ad-CSP +Ad-GFP. Splenocytes were collected from 7 7 BALB/cJ mice 14 days post co-injection of either 5x10 vps of Ad-CSP and 5x10 vps of Ad7 7 6 GFP or 5x10 vps of Ad-CSP and 5x10 vps of Ad-EAT2. 2×10 splenocytes from naive or mice co-vaccinated with either treatment were stimulated with 2ug NYD-peptide at 37°C for 3 days. Cells were then washed with FACS buffer and stained with CD8-Alexa700, CD107-FITC + + antibodies and viability dye (ViViD) and ran on LSR-II. % of live CD107 CD3 T cells is shown. The bars represent mean ± SD. Statistical analysis was completed using One Way ANOVA with a Student-Newman-Keuls post-hoc test,* Indicates significance over naïve p<0.05 124 Figure 26: Improved degranulation of CD8+ T cells in mice co-vaccinated with Ad-CSP and Ad-EAT2. 125 We then conducted a more sensitive in vivo CTL assay (362). Mice were co-vaccinated 8 8 8 with either 1x10 vp/mouse of Ad-CSP and 1x10 vps/mouse of Ad-GFP or 1x10 vp/mouse of 8 Ad-CSP and 1x10 vps/mouse of Ad-EAT2. 14 days later vaccinated mice were treated with CFSE labeled splenocytes that had been incubated with either the NYDNAGTNL peptide, or a non-specific control peptide, and the elimination of NYDNAGTNL pulsed cells (CFSE high cells) was measured by flow cytometry. Based on the calculated percent specific killing, mice vaccinated with Ad-CSP+Ad-EAT2 were more effective at killing cells exposed to the NYDNAGTNL peptide than mice vaccinated with the control Ad-CSP vaccine (Figure 27). Figure 27: Co-expression of CS protein and EAT-2 increases cytolytic activity of CS protein specific T cells. Co-vaccination of mice with Ad-CSP and Ad-EAT2 increased specific killing cells pulsed with CS protein peptides. BALB/cJ mice (n=4) were co-injected IM with 8 8 8 either 1x10 vps/mouse Ad-CSP and 1x10 vps/mouse Ad-GFP or 1x10 vps/mouse Ad-CSP 8 and 1x10 vps/mouse Ad-EAT2 on Day 0. Day 14 splenocytes were collected from naïve mice and pulsed with either NYDNAGTNL peptide or an irrelevant peptide. NYDNAGTNL pulsed splenocytes were stained with a high concentration of CFSE while splenocytes pulsed with irrelevant peptide were stained with a low concentration of CFSE. Stained splenocytes were then combined in equivalent doses. 8 million cells were then injected IV into naïve, Ad-CSP + AdGFP co-vaccinated, or Ad-CSP+Ad-EAT2 co-vaccinated mice. After 18 hrs splenocytes from these mice were collected and analyzed by flow cytometry to assess the amount of NYDNAGTNL specific killing. 126 Figure 27: Co-expression of CS protein and EAT-2 increases cytolytic activity of CS protein specific T cells. 127 CS protein antibody specific ELISAs were also performed on plasma derived from AdCSP +Ad-GFP/rEA and Ad-CSP +Ad-GFP treated mice. CS protein specific total IgG antibody levels in control vaccine treated animals were significantly elevated (p<0.05) as compared to naïve animals. However, there was again no significant difference observed in Ad-CSP +AdGFP/rEA treated animals when compared to naïve animals (p<0.05) (Figure 28A). Conversely, plasma collected from Ad-CSP+Ad-EAT2 treated animals had significantly higher levels of CS protein specific IgG as compared to levels detected in naïve mice (p<0.05) (Figure 28B). However, the mice receiving the control vaccine treatment had higher total CS protein specific IgG levels than naïve and Ad-CSP +Ad-EAT2 treated animals (p<0.05) (Figure 28B). Figure 28: Induction of CS protein specific antibody responses by Ad-CSP vaccines augmented by rEA or EAT-2. Total IgG antibody against CS protein is ablated in Ad-CSP +Ad-GFP/rEA co-vaccinated mice while Ad-CSP+Ad-EAT2 co-vaccinated mice demonstrated significantly more CS protein specific IgG than naïve animals. BALB/cJ mice (n=5) were co7 injected IM with 5x10 vps/mouse of Ad-CSP and 5x107 vps/mouse of Ad-GFP or 5x10 7 7 vps/mouse of Ad-CSP and 5x10 vps/mouse of either (A) Ad-GFP/rEA or (B) Ad-EAT2. Plasma was collected at day 14. Total IgG against CS protein in the plasma was measured by ELISA. The bars represent mean ± SD. Statistical analysis was completed using One Way ANOVA with a Student-Newman-Keuls post-hoc test, * Denotes significance over naïve p<0.05. † Denotes significant difference between treatments p<0.05. 128 Figure 28: Induction of CS protein specific antibody responses by Ad-CSP vaccines augmented by rEA or EAT-2. 129 Further isotyping of IgG was performed, the ratios of Th1 to Th2 antibody (IgG2a/IgG1) in mice treated with Ad-EAT2 +Ad-CSP were similar to the ratio of Th1 to Th2 antibody in control treated mice in all dilution except 1:400, indicating that expression of EAT-2 did not induce a Th1 to Th2 bias in these mice at 14 dpi as measured by this assay (Figure 29). Figure 29: Sub-isotype analysis of IgG antibody from plasma of mice co-vaccinated with 7 Ad-CSP and Ad-EAT2. BALB/cJ mice (n=6) were co-injected i.m. with 5x10 vps of Ad-CSP 7 7 7 and 5x10 vps of Ad-GFP or 5x10 vps of Ad-CSP and 5x10 vps of Ad-EAT2. Plasma was collected at day 14. (A) The amount of CS protein specific IgG sub-isotypes was measured by ELISA. (B) The ratio of IgG2a/IgG1 was calculated to indicate the Th1 to Th2 response ratio. The bars represent mean ± SD. Statistical analysis for sub-isotyping (A) was completed using One Way ANOVA with a Student-Newman-Keuls post-hoc test and standard t-test was performed for Th1 to Th2 ratios (B),* indicates significance over naïve p<0.05. † Indicates significance between treatments p<0.05. 130 Figure 29: Sub-isotype analysis of IgG antibody from plasma of mice co-vaccinated with Ad-CSP and Ad-EAT2. 131 In addition, when measured by intracellular staining, there was no significant difference + - + in the number of likely CD4 IFNγ expressing T-cells, as the number of CD8 CD3 T cells in Ad-CSP +Ad-EAT2 treated animals and were similar to the numbers of these cells noted in AdCSP +Ad-GFP treated animals (Figure 30). + - + Figure 30: CD3 CD8 IFNγ cells respond similarly to both vaccine regimens. Co+ - vaccination with Ad-CSP and Ad-EAT2 resulted in similar IFNγ secretion from CD3 CD8 T 7 cells. BALB/cJ mice (n=6) were co-injected IM with 5x10 vps/mouse of Ad-CSP and 5x10 7 7 7 vps/mouse of Ad-EAT2 or 5x10 vps/mouse of Ad-CSP and 5x10 vps/mouse of Ad-GFP. Splenocytes were stimulation with NYDNAGTNL peptide. Cells were stained with CD8-Alexa Flour700, CD3-APC-Cy7, ViViD, and IFNγ-APC. The bars represent mean ± SD. Statistical analysis was completed using One Way ANOVA with a Student-Newman-Keuls post-hoc test. 132 + - + Figure 30: CD3 CD8 IFNγ cells respond similarly to both vaccine regimens. 133 3.3. Discussion: Our earlier works and those of others suggest that activation of the innate immune system can play an important role in beneficially augmenting subsequent antigen specific adaptive immune responses (179, 184, 363-365). For example, we previously augmented CMI responses against HIV-Gag by co-injecting a rAd5 vector expressing HIV-Gag with a rAd5 vector expressing a TLR agonist, rEA (363). Similarly, co-injecting a rAd5 vector expressing HIV-Gag with a rAd5 vector expressing the SLAM receptors adaptor protein EAT-2 also augmented induction of innate immune responses, and improved the induction of HIV-Gag specific T cell responses (358). As a new approach to increasing the potency of malaria specific vaccines, we now describe the use of adenoviral based vaccines engineered to express malaria derived proteins, simultaneously administered with rAds expressing proteins known to modulate the innate immune system. Most importantly, we have confirmed that rAd mediated expression of a SLAM pathway derived adaptor (EAT-2) can significantly augment the induction of malaria antigen (CS protein) specific CMI responses. This was verified based upon ELISpost analysis of splenocytes (both as to their responsiveness to immunodominant peptides, as well the breadth of these responses to the full length CS protein), ICS staining of cells for IFNγ, and most importantly by a CS protein specific in vivo CTL functional assay. EAT-2 expressing vaccines should be considered for use in future malaria vaccine trials attempting to boost malaria antigen specific CMI responses. Furthermore, EAT-2 co-expression allowed for the induction of CS specific antibody responses as well. In contrast, co-vaccination of mice with a rAd vaccine expressing a TLR agonist simultaneously with a rAd expressing CS protein, actually had the opposite effect, and completely mitigated induction of CS protein specific adaptive humoral and cellular immune 134 responses, as compared to responses typically induced by the rAd vaccines expressing CS protein alone. There could be numerous reasons for these unexpected, paradoxical and potentially disturbing results. A simple reason could be that the increase in pro-inflammatory cytokines caused by rAd mediated expression of the TLR agonist, rEA, could be influencing expression of CS protein from the rAd5 vector. However, this effect would have likely been observed in our previous studies utilizing the same vector combinations, as well as the same TLR or SLAM receptors derived adaptors, but a different target antigen (HIV-Gag). Those studies also confirmed induction of similar innate immune responses to those noted in this study (358, 363). It is more logical that the CS protein somehow negatively interacts with immune pathways excessively activated by TLR agonists such as rEA, resulting in a complete ablation of CS protein specific CMI responses. This immunosuppressive activity of CS protein appears to only be unveiled after excessive stimulation of TLR pathways, as our use of EAT-2 demonstrated not only avoidance of CS immunosuppressive activity, but also allowed for enhanced induction of CS specific adaptive immune responses. The CS protein has been specifically confirmed to be capable of outcompeting the transcription factor NF-κB for binding to the nuclear transport protein, importan α, resulting in the down-regulation of at least forty NF-κB controlled genes (366). CS protein was also shown to inhibit NF-κB entry into the nucleus by 75% (366). As NF-κB is known to control numerous genes involved in pro-inflammatory immune responses, one hypothesis may be that the CS protein can downregulate excessive (TLR-driven) NF-κB transcriptome responses, and result in a dramatically diminished acute inflammatory response, thereby blunting subsequent CS protein antigen specific adaptive immune responses (367). This may make biological sense, as infection of hepatocytes by malaria sporozoites has been shown to induce the activation of NF-κB in a 135 MyD88 specific manner (368). Expression of CS protein by the parasite may have evolved to counteract this inflammatory response and prevent excessive induction of malaria specific adaptive immune responses in the infected host. Interestingly, recent studies on an immunosuppressive drug (dehydroxymethylepoxyquinomicin) that specifically interferes with the NF-κB-importan α interaction was shown at lower doses to only modestly affect IL-6 and TNFα levels, while dramatically affecting Th1 expansion, results paralleling those noted in our experiments (369). These notions may also explain our findings, as well results previously reported by others (348, 359). Those studies and ours verify that at very high doses, rAd vaccines expressing CS protein also show a trend toward diminished induction of CS protein specific CMI responses (Figure 18) (348, 359). Multiple studies have shown that Ad vectors can also induce NF-κB (181, 370). Quite possibly, the CS protein immunosuppressive effects are not uncovered until an “NF-κB activation threshold” has been broached, in this instance by use of excessively high doses of rAd vaccines expressing CS protein, or by using more modest doses of the Ad vaccine coupled with potent TLR activation. Further studies will need to be performed to elucidate whether this or other mechanisms may be responsible for our results. Regardless, our data demonstrate the need to consider the impact the inclusion of CS protein derived peptides, or the entire protein along with other immunostimulatory compounds may have upon present and future malaria specific vaccines. Taken together with recent data demonstrating that protection from malaria challenge can be independent of CS protein suggests that the use of CS protein in certain malaria vaccine formulations will have to be carefully considered (371, 372). In contrast to co-expression of the TLR agonist, co-expression of EAT-2 and CS protein eventuated in the enhanced induction of CMI responses to the CS protein, relative to the use of 136 the Ad-CSP vaccine alone. We have also previously observed a potent CMI response against HIV derived Gag in mice treated with Ad-EAT2 +Ad-Gag (358). Like TLRs, activation of the SLAM receptor pathway in DCs and macrophages can also enhance the production of proinflammatory cytokines (373). The biochemical mechanism and intracellular signaling pathway behind EAT-2’s ability to function as a T cell (and possibly a B cell) stimulator in the face of CS protein over expression is not fully elucidated, but is a question that has been unveiled by our studies. SLAM associated proteins like EAT-2 are known to play a role in several novel immuno-modulatory pathways, including the SLAM, CD22, and FcγRIIB (374-376). These pathways may not be subject to the immune suppressive actions of CS protein possibly by virtue of its specific mode of action relative to NF-κB and/or TLR activation pathways described earlier. + It has been established that greater numbers of CD8 T cells are required to police infected hepatocytes and achieve long term protective immunity against malaria, emphasizing + the importance of inducing a large population of CD8 T cells capable of killing (377). There is some evidence that improved protection is also related to increased breadth of the CMI response in addition to the potency of the CMI response (309, 361, 378). Here, as an accessory to the increased CMI response, we have demonstrated Ad-EAT2’s ability to stimulate increased T cell responses against multiple CS protein epitopes. We not only observed an increase in the + percentage of CS protein specific CD8 T cells, but also improved in vivo CTL killing of CS pulsed splenocytes from mice treated with Ad-CSP +Ad-EAT2. The use of EAT-2 to augment + CSP specific functional CD8 T cells may be of greatest importance in killing Plasmodium infected hepatocytes, as these types of responses are not only positively correlated with 137 protective capability, but also may outweigh the need for induction of malaria antigen specific antibody responses (337, 338, 377, 379). Improvements over sole use of Ad-CSP to induce CS protein antigen specific B cell responses were not achieved in mice treated with either Ad-CSP vaccine cocktail. However, covaccination with the Ad-CSP and Ad-EAT2 vectors at least prevented the loss of induction of CS specific antibody responses noted after use of the Ad-GFP/rEA and Ad-CSP vaccine combination. These results did not appear to be due to a skewing from Th1 to Th2 type antibody response, as measured by IgG1/IgG2a ratios; there were also no observed differences in IFNγ - + secreting CD8 CD3 T cells between treatment groups. Further research will need to be performed to elucidate the reasons behind the observed antibody responses. The importance of stimulating a strong cytotoxic T cell response against P. falciparum infected hepatocytes is vital in creating a subunit based vaccine that is protective against malaria. With this study we have successfully stimulated a CMI response to CS protein that can overcome CS protein related adaptive immune response ablation and is even more potent than the previous generation of rAd5s expressing CS protein. Incorporation of this new vaccine platform into ongoing or future malaria vaccine trials could potentially achieve the levels of prophylaxis needed to protect vulnerable populations against natural malaria infections. Future studies will need to be performed to assess this platforms ability to protect larger animals challenged with malaria. 138 Acknowledgements: We wish to thank Michigan State University Laboratory Animal support facilities for their assistance in the humane care and maintenance of the animals utilized in this work, the NIH Core Tetramer Facility at Emory University for manufacturing the NYDNAGTNL tetramer, and the Michigan State University flow cytometry facility for their assistance with the multiple experiments. A.A. was supported by the National Institutes of Health grants RO1 AR056981 and P01 CA078673, the MSU Foundation, as well the Osteopathic Heritage Foundation. YAA was supported by the King Abdullah bin Abdulaziz Scholarship, Ministry of Higher Education, Kingdom of Saudi Arabia. 139 Chapter IV TRIF is a critical negative regulator of TLR agonist mediated activation of dendritic cells in vivo. This chapter is the edited version of a research article that was published in PLoS ONE Journal, Volume 6, Issue 7 (e22064), July 8, 2011. Authors: Aldhamen Y.A.*, Seregin S.S. *, Appledorn D.M., Aylsworth C.F., Godbehere S., Liu C.J., Quiroga D., and Amalfitano A. *Authors contributed equally to the contents of this paper. 140 4.1. Introduction There is a great need to develop more efficient vaccines to combat or prevent infections by a number of detrimental pathogens that continue to plague mankind (380-382). The use of novel adjuvants capable of beneficially stimulating the immune system to maximize efficacy of various vaccination strategies is a rapidly developing field. Most adjuvants augment the induction of innate immune responses by triggering robust activation of dendritic cells (DCs) and macrophages, actions that can result in improved induction of antigen specific adaptive immune responses. Upon migration to the draining lymph nodes, these highly active antigen presenting cells (APCs) are capable of presenting specific antigens to responsive T cells, thereby generating significant pools of antigen-specific T cells (381, 383). Incorporation of Toll-like receptor (TLR) ligands into vaccine formulations represent a class of adjuvants proposed for usage in next generation vaccines. This is primarily due to TLRs being expressed at high levels on important immune cell types (DCs, macrophages, NK cells) and their ability to potently activate the innate immune system (383-385). In light of these facts, the recombinant, Eimeria tenella derived antigen (rEA) has been proven to be capable of inducing IL-12p70 production, enhancing Th1 cellular responses, and yielding protection against Toxoplasma gondii infection in mice (134). rEA has also been shown to be an efficient immunomodulator, having both antiviral and anti-cancer properties (131, 135, 136). Previous studies have also shown that HIV-Gag-specific T cell responses are significantly increased when rEA formulations are administered together with the antigen (133, 139). Moreover, rEA showed no evidence of toxicity in pre-clinical (386) and clinical trials (137). Specifically, no severe adverse reactions were reported in human clinical trials despite detection of increased IL-12 responses in 30% of the treated cancer patients (137). 141 The rEA protein has a relatively high amino acid sequence homology (67%) and shares very similar biological activities in vitro and in vivo with T. gondii-derived profilin-like protein, both of which trigger potent IL-12 responses in DCs. The profilin induced responses were completely dependent upon the adaptor protein MyD88 and at least partially mediated via TLR11 (138). Moreover, it has been shown in vitro that human TLRs (TLR2, TLR3, TLR4, TLR5, TLR7, TLR8 and TLR9) do not transduce rEA signaling (135). Therefore, TLR11 has been suggested as the rEA receptor mediating rEA signaling, but this notion remains to be confirmed. Since no functional human TLR11 homolog has been discovered, these facts leave unidentified the mechanism underlying rEA action in humans, and opens a discussion regarding other pattern recognition receptors (PRRs) that may be involved in rEA signaling (135). Additionally, it is not known what cell types are primarily responsible for mediating rEAtriggered responses in vivo. MyD88 and TRIF are two adaptor proteins which primarily mediate the signaling derived from activation of many pattern recognition receptors (PRRs), including TLRs (385). We have investigated if rEA requires either of these two proteins to trigger immune responses in vivo, and have found that all rEA-triggered immune responses are dependent on MyD88 functionality (including the rapid activation of DCs, macrophages, NK, NKT, T and B cells, the induction of pro-inflammatory cytokine/chemokine releases, as well as Erk1/2 phosphorylation). Surprisingly, we discovered that functional TRIF protein acts to suppress rEA induction of these same responses; thereby unveiling a novel inhibitory role for TRIF during rEA mediated signaling. We also present evidence that TRIF may similarly suppress TLR activations by other known TLR ligands. Together the findings highlight the complexities underlying adjuvant activation of the innate immune system, as well suggests that simple notions of augmenting or 142 modifying adjuvant activity by use of TLR system agonists or antagonists may be complicated by these complex molecular mechanisms. 143 4.2. Results The purpose of this study was to identify the impact of rEA stimulation on host immune systems, to define important cell types that respond to or modulate rEA-driven activation, and to identify signaling pathways responsible for activation of the immune system in response to rEA. To investigate this, C57BL/6 mice were each intraperitoneally (IP) injected with 100 ng of purified, rEA protein. Splenocytes were harvested at 6 hpi and flow cytometry was performed as + detailed in Materials and Methods. We identified that activation of murine DCs (CD11c , - - - CD11b , CD19 , CD3 ) in response to rEA was completely dependent on the presence of full + MyD88 functionality. Specifically, we found significant increases in the percent of CD40 , + + CD80 and CD86 DCs (as well as induction of the expression of these molecules per cell as measured by Mean Fluorescent Intensity [MFI]) in rEA treated wild type (WT) mice, but no such increases were observed in rEA treated MyD88 knockout (KO) or MyD88/TRIF double knockout (DKO) mice, each as compared to mock-injected animals. Surprisingly, we detected significant (p<0.001) increases in DC activation after identical rEA treatments of TRIF-KO mice (as compared to WT mice), as measured by CD40, CD80 and CD86 surface staining. Furthermore, the amount of CD86 expression per cell was significantly (p<0.001) increased in rEA treated TRIF-KO mice, as compared to rEA treated WT mice (Figures 31). rEA stimulation also increased the percent of MHC-II presenting DCs in WT and TRIF-KO mice, increases that were not seen in the rEA-treated MyD88-KO or the MyD88/TRIF-DKO mice. The DCs from rEA-treated TRIF-KO mice also had significantly (p<0.05) higher MHC-II surface expression levels as compared to rEA treated WT mice (Figure 31). 144 Figure 31: TRIF acts as a negative regulator of rEA-induced MyD88-dependent activation of dendritic cells in vivo. C57BL/6 WT (N=3-4), MyD88-KO (N=3), TRIF-KO (N=3-4), and MyD88/TRIF-DKO (N=4) mice were injected with 100 ng of rEA. Splenocytes were harvested at 6 hpi, processed, stained for expression of surface markers, and FACS sorted as described in Materials and Methods. All genotype mock-injected mice (N=2-3) were included in analysis. One of two representative experiments is shown. Separate sets of WT mice were utilized for + - - comparison with each knockout genotype. Activation of CD11c , CD19 , and CD3 DCs is shown. The bars represent Mean ± SEM. Statistical analysis was completed using a two tailed homoscedastic Student’s t-tests. *, ** - Indicate values statistically different from those in mock injected animals (of the same genotype), p<0.05, p<0.001 respectively. 145 TRIF acts as a negative regulator of rEA-induced MyD88-dependent activation of dendritic cells in vivo. 146 + - - rEA-mediated activation of splenic macrophages (CD11b , CD19 , CD3 ) was also completely dependent on MyD88, as confirmed by lack of macrophage activation in response to rEA stimulation in the MyD88-KO or the MyD88/TRIF-DKO mice. In contrast, WT mice injected with rEA experienced a dramatic increase in the levels of CD80, CD86, and MHC-II, on the surface of splenic macrophages (MFI), as well as in the percent of macrophages, expressing the CD40, CD80, and CD86 activation markers. Similar to observations in DCs, macrophages derived from rEA-treated TRIF-KO mice were activated to levels that were significantly higher than levels measured in WT mice treated with rEA. Not only were the amounts of CD40expressing macrophages increased, but also both the percentages of CD80 and CD86 expressing macrophages (p<0.01) and amount of these markers per cell (MFI, p<0.05) were significantly increased in rEA treated TRIF-KO mice as compared to rEA-treated wild type mice, (p<0.05) (Figures 32). These experiments unveiled an important, not previously described, role of TRIF as a suppressor of rEA-induced TLR/MyD88 signaling in DCs and macrophages in vivo. Figure 32: TRIF acts as a negative regulator of rEA-induced MyD88-dependent activation of macrophages in vivo. C57BL/6 WT (N=3-4), MyD88-KO (N=3), TRIF-KO (N=3-4), and MyD88/TRIF-DKO (N=4) mice were injected with 100 ng of rEA. Splenocytes were harvested at 6 hpi, processed, stained for expression of surface markers, and FACS sorted as described in Materials and Methods. All genotype mock-injected mice (N=2-3) were included in analysis. One of two representative experiments is shown. Separate sets of WT mice were utilized for comparison with each knockout genotype. Activation of macrophages is shown. The bars represent Mean ± SEM. Statistical analysis was completed using a two tailed homoscedastic Student’s t-tests. *, ** - Indicate values significantly higher (#, ## - lower) from those in mock injected animals (of the same genotype), p<0.05, p<0.001 respectively. 147 Figure 32: TRIF acts as a negative regulator of rEA-induced MyD88-dependent activation of macrophages in vivo. 148 Interestingly, we found that baseline levels of MHC-II expression on splenic DCs were significantly increased in untreated, MyD88-KO and MyD88/TRIF-DKO mice (amount of MHCII per cell, p<0.01, Figure 22) as compared to untreated WT mice or untreated TRIF-KO mice (Figures 31,32 and 33). Additionally, the MyD88/TRIF-DKO mice also had higher baseline levels of CD40 expression in DCs (Figure 33). This phenomenon was not unexpected, as we had previously noted increased baseline levels of the MHCII -chain (three-fold higher) in MyD88KO mice, as confirmed by microarray transcriptome analysis and flow cytometry based analyses ((181) and data not shown). Potentially, these baseline changes may be due to lack of presence of these adaptors during normal mouse development. Figure 33: TRIF acts as a negative regulator of rEA-induced MyD88-dependent activation of dendritic cells in vivo (MFI). C57BL/6 WT (N=3-4), MyD88-KO (N=3), TRIF-KO (N=3-4), and MyD88/TRIF-DKO (N=4) mice were injected with 100 ng of rEA. Splenocytes were harvested at 6 hpi, processed, stained for expression of surface markers, and FACS sorted as described in Materials and Methods. All genotype mock-injected mice (N=2-3) were included in analysis. One of two representative experiments is shown. Separate sets of WT mice were utilized for comparison with each knockout genotype. Mean Fluorescent Intensity (MFI) is shown and is indicative of amount of analyte per cell. The bars represent Mean ± SEM. Statistical analysis was completed using two-tailed homoscedastic Student’s t-tests. *, ** Indicate values statistically different from those in mock-injected animals (of the same genotype), p<0.05, p<0.001 respectively. 149 Figure 33: TRIF acts as a negative regulator of rEA-induced MyD88-dependent activation of dendritic cells in vivo (MFI). 150 The unexpected result of TRIF being a negative regulator of rEA-triggered activation of DCs and macrophages, prompted us to evaluate if TRIF reduces rEA-induced activation of other important immune cells, including effector NK cells, as well as NKT, T and B cells. We have previously shown that rEA protein activates splenic and hepatic NK and NKT cells within 6 hours post-injection in WT mice (133). In this study, rEA injections into MyD88-KO and MyD88/TRIF-DKO mice yielded only baseline activation levels of CD69+ NK, NKT, T and B cells. This contrasted with a significant activation of these same cell types in WT mice treated with rEA protein (Figure 34). Similarly, the amount of IFN production from NK and NKT cells was not increased in the MyD88-KO or the MyD88/TRIF-DKO mice in response to rEA, whereas WT mice had dramatically increased numbers of IFN secreting NK (from 1% to 2030%) and NKT (from 1% to 2-5%) cells. In contrast, rEA treatment of TRIF-KO mice revealed significant increases in the number of IFN producing NK cells (p<0.05), the amount of CD69 expression per NK cell (MFI, p<0.01), the number of cells (p<0.05) and amount (p<0.05) of IFN-production from NKT cells, the number of cells (p<0.01) and amount (p<0.01) of CD69 expression from T cells, and the number of B cells expressing CD69 (p<0.01); all in comparison to rEA-treated WT mice (Figure 34). Figure 34: TRIF acts as a negative regulator of rEA-induced MyD88-dependent activation of NK, NKT, T, and B cells in vivo. C57BL/6 WT (N=3-4), MyD88-KO (N=3), TRIF-KO (N=3-4), and MyD88/TRIF-DKO (N=4) mice were injected with 100 ng of rEA. Splenocytes were harvested at 6 hpi, processed, stained for expression of surface markers (intracellular staining was performed for IFN), and FACS sorted as described in Materials and Methods. All genotype mock-injected mice (N=2-3) were included in analysis. Separate sets of WT mice were 151 utilized for comparison with each knockout genotype. Activation of NK, NKT, T, and B cells is shown. The bars represent Mean ± SEM. Statistical analysis was completed using a two tailed homoscedastic Student’s t-tests. *, ** - Indicate values statistically different from those in mock injected animals (of the same genotype), p<0.05, p<0.001 respectively. 152 Figure 34: TRIF acts as a negative regulator of rEA-induced MyD88-dependent activation of NK, NKT, T, and B cells in vivo. 153 Within 6 hours of administration, rEA protein triggers significant production of proinflammatory cytokines and chemokines in mice. Specifically, circulating levels of IL12p70, IL6, TNF, IFN, and IL2 were markedly elevated in rEA-treated mice (135, 386). Purified murine DCs exposed to 0.2 ng/ml of rEA were shown to release significant amounts of IL12p70, IL6, and IL2 (386). We confirmed and extended the observation that rEA triggers the release of a wide spectrum of pro-inflammatory cytokines and chemokines, including IL6, IL12p40, IL12p70, GCSF, IFN, IL2, IL1, IL1, IL10, IL13, GMCSF, KC, MCP1, MIP1, MIP1, RANTES, and TNF. This effect was however, completely dependent on MyD88 given that all of these analytes were induced in rEA-treated WT, but not in rEA-treated MyD88-KO or MyD88/TRIF-DKO mice (Figure 35). Again, paralleling our previous results, the release of all of these cytokines and chemokines was significantly increased in rEA-treated TRIF-KO mice, and the majority of these analytes were elevated to levels that were significantly higher than levels measured in rEA-treated WT mice. In particular, IL6 was induced to ~3 fold (p<0.001) higher levels and IL12p40, GCSF, and IFN were induced to over 2 fold (p<0.001) higher levels when comparing rEA-treated TRIF-KO mice to rEA-treated WT mice. Moreover, IL12p70, IL2, IL1, IL1, and MIP1 were also produced at significantly higher levels in rEA-treated TRIFKO mice as compared to rEA treated WT mice (Figure 35). Figure 35: TRIF negatively regulates rEA-mediated MyD88 dependent activation of proinflammatory cytokines and chemokines in vivo. C57BL/6 WT (N=9), MyD88-KO (N=3), TRIF-KO (N=3-4), and MyD88/TRIF-DKO (N=4) mice were injected with 100 ng of rEA. Plasma samples were collected at 6 hpi and were analyzed using a multiplexed bead array based quantitative system. All genotype mock-injected mice (N=2-3) were included in analysis. One of two representative experiments is shown. Statistical analysis was completed using a one-way 154 ANOVA with a Student-Newman-Keuls post-hoc test. The bars represent Mean ± SD. *, ** Indicate plasma cytokine values that are statistically different from those in mock injected animals, p<0.05, p<0.001 respectively. No significant differences between mock-injected animals of different genotypes were detected. No significant activation of cytokines was observed in MyD88-KO and MyD88/TRIF-DKO animals. 155 Figure 35: TRIF negatively regulates rEA-mediated MyD88 dependent activation of proinflammatory cytokines and chemokines in vivo. 156 It is known that many of the rEA induced cytokines and chemokines are released by DCs + (Figure 35) (387). For that reason, we purified CD11c DCs from WT, MyD88-KO, TRIF-KO, and MyD88/TRIF-DKO mice, and then stimulated the cells ex vivo with escalating amounts of rEA protein. Utilizing an IL12p70 specific ELISA, we confirmed that in response to rEA, DC production of this cytokine was completely dependent upon functional MyD88 (Figure 36A). The minimal rEA dose in which WT mouse-derived DCs produced significant amounts of IL12p70 was found to be 100 pg/ml, whereas TRIF-KO mouse-derived DCs only required a 10 pg/ml dose (10 fold less) for significant IL12p70 release. In an overall comparison to WT mouse derived DCs, TRIF-KO mouse derived DCs had a significantly higher production of IL12p70 in response to rEA. The most dramatic difference between these two groups was noted when DCs were stimulated with a 0.2 ng/ml dose of rEA, which caused ~1300 pg/ml of IL12p70 to be released from DCs derived from TRIF-KO mice as compared to ~600 pg/ml from DCs derived from WT mice (p<0.01) (Figure 36A.). We have verified our ELISA-based data for IL12p70 independently, by Bioplex bead array. Again, we confirmed that MyD88-KO and the MyD88/TRIF-DKO mouse derived DCs each respectively failed to produce significant levels of pro-inflammatory cytokines and chemokines in response to rEA stimulation. Interestingly, we also confirmed that these rEA mediated DC responses were also partially suppressed by TRIF, as, IL2, IL6, IL12p40, IL12p70, IL1, IL1, and MIP1 were released to significantly (p<0.05) higher levels in rEA-treated DCs derived from TRIF-KO mice, as compared to those derived from WT mice (Figure 36B). Figure 36: TRIF negatively regulates rEA-mediated MyD88 dependent activation of pro+ inflammatory cytokines and chemokines in dendritic cells. (A) CD11c dendritic cells were isolated from C57BL/6 WT (N=2), MyD88-KO (N=2), TRIF-KO (N=2), and MyD88/TRIF157 DKO (N=2) mice, in vitro stimulated with rEA, then used to perform a IL12p70 ELISA as described in Materials and Methods. One (of three) representative experiments is shown. The bars represent Mean ± SD. Statistical analysis was completed using a two-way ANOVA with a Bonferroni post-hoc test (genotypes x rEA treatments). *, ** - Indicate values that are statistically different from those in unstimulated DCs (for the same genotype), p<0.05, p<0.001 respectively. #, ## - Indicate values statistically different from those in WT DCs (for the same rEA dose), p<0.05, p<0.01 respectively. No significant differences between mock-injected animals of different genotypes were detected. No significant activation of IL12p70 was observed in MyD88-KO and MyD88/TRIF-DKO DCs. (B) DC culture media was collected at 18 hours post-rEA stimulation (0.2 ng/ml) and was analyzed for cytokines/chemokines levels using a multiplexed bead array based quantitative system. Statistical analysis was completed using a one-way ANOVA with a Student-Newman-Keuls post-hoc test. The bars represent Mean ± SD. *, ** - Indicate cytokine values that are statistically different from those in mock injected animals, p<0.05, p<0.001 respectively. No significant differences between mock-injected animals of different genotypes were detected. No significant activation of pro-inflammatory cytokines was observed in MyD88-KO and MyD88/TRIF-DKO animals (only anti-inflammatory IL10 cytokine was induced in MyD88-KO mice). 158 Figure 36: TRIF negatively regulates rEA-mediated MyD88 dependent activation of pro-inflammatory cytokines and chemokines in dendritic cells. 159 To more fully investigate if TRIF suppressive effects were rEA-specific or a more global phenomenon, we have specifically stimulated CD11c+ DCs, isolated from WT, TRIF-KO, MyD88-KO or MyD88/TRIF-DKO mice with various common TLR4, TLR7/8, and TLR9 agonists. Specifically, LPS, R848 and ODN2006 were utilized in these experiments. All of these TLR agonists were able to induce pro-inflammatory cytokine production after administration to DCs, as compared to unstimulated DCs, all derived from WT mice. Importantly, however, DCs derived from TRIF-KO mice had dramatically higher levels of secretion of pleiotropic proinflammatory cytokines, when stimulated with these same TLR agonists (Figure 37AB). Specifically, ODN2006 stimulation resulted in significantly higher IL6, IL12p40, IL12p70 and MIP1 levels; LPS stimulation significantly increased IL1, IL3, IL6, IL12p70 levels; R848 treatment resulted in significantly higher production of IL1, IL1, IL6, and MIP1; in DCs, derived from TRIF-KO mice as compared to DCs derived from WT mice, as tested both by ELISA (Figure 37A) or Bioplex analysis (Figure 37B and data not shown). DCs derived from MyD88-KO or MyD88/TRIF-DKO mice did not show any significant cytokine activations when stimulated with any of these TLR agonists (data not shown). Interestingly, lack of the TRIF adaptor protein resulted in up to 2 fold increases in cytokine production from DCs, when stimulated with LPS or ODN2006 (i.e. IL12p70), indicating that the suppressive role of TRIF in this cell type might be immunologically significant. Figure 37: TRIF negatively regulates cytokine production by DCs, triggered by several + common TLR agonists. (A) CD11c dendritic cells were isolated from C57BL/6 WT (N=3), MyD88-KO (N=3), TRIF-KO (N=3), and MyD88/TRIF-DKO (N=3) mice, in vitro stimulated with various TLR agonists, and used to perform a IL12p70 ELISA as described in Materials and Methods. The bars represent Mean ± SEM. Statistical analysis was completed using a one-way 160 ANOVA with Student-Newman-Keuls post-hoc test. ** - Indicate values that are statistically different from those in unstimulated DCs (for the same genotype), p<0.001. No significant differences between mock-injected animals of different genotypes were detected. No significant activation of IL12p70 was observed in MyD88-KO and MyD88/TRIF-DKO DCs. (B) DC culture media was collected at 15 hours post stimulation with various TLR agonists (rEA, LPS, ODN2006) and was analyzed for cytokines/chemokines levels using a multiplexed bead array based quantitative system. Statistical analysis was completed using a one-way ANOVA with a Student-Newman-Keuls post-hoc test. The bars represent Mean ± SD. *, ** - Indicate cytokine values that are statistically different from those in mock injected animals, p<0.05, p<0.001 respectively. 161 Figure 37: TRIF negatively regulates cytokine production by DCs, triggered by several common TLR agonists. 162 Extensive past research has demonstrated that the major signaling pathways activated downstream of TLR adaptor proteins (e.g. MyD88, TRIF) are the NFB and MAPK pathways (8). Along with many other biological roles, these pathways promote production of cytokines and chemokines as well as proliferation, maturation, and development of various immune cells. To identify which of the several signaling pathways may be activated in response to rEA in vivo, we IP injected WT or MyD88-KO mice with 100 ng of rEA, and then collected spleen and liver tissues at various time points (0-120 minutes) post-injection. We found that rEA-triggered Erk1/2 phosphorylation was MyD88 dependent, as confirmed by increased Erk1/2 phosphorylation in rEA-treated WT mice, but not in the MyD88-KO mice (Figure 38). Figure 38: rEA-triggered Erk1/2 phosphorylation is MyD88 dependent. C57BL/6 WT or MyD88-KO mice were injected with 100 ng of rEA. Spleen (A) and liver (B) tissues were collected at the indicated time points and processed as described in Materials and Methods. pErk1/2 and Erk2 levels were determined by Western blot analysis using LI-COR Odyssey. To control for loading, quantification was performed after normalizing the pErk1/2 to Erk2 levels. Three independent experiments representative of this data are shown. (C) Representative blots: spleen (top), liver (bottom). 163 Figure 38: rEA-triggered Erk1/2 phosphorylation is MyD88 dependent. C57BL/6 WT or MyD88-KO mice were injected with 100 ng of rEA. 164 4.3. Discussion The ideal adjuvant (from Latin “adjuvare”, meaning “to enhance”) is an agent that is capable of dramatically enhancing both cellular and humoral adaptive immune responses to coadministered antigens, thereby providing more efficient and long-term protection against specific pathogens. Aluminum salts, discovered to be potent adjuvants in 1920s, remained the only FDA approved adjuvant for many decades and still represent one of the few in clinical use. A barrier in adjuvant research has been that the mechanism of action of many adjuvants remained poorly understood. Earlier studies showed that alum and squalene-based emulsion MF59 promote recruitment and increase antigen uptake by APCs, induce cytokine and chemokine secretions, and the expression of adhesion molecules involved in migration of leukocytes (388). More recently it has also been confirmed that alum and squalene based adjuvants may use the NODlike receptor protein 3 (NLRP3) inflammasome pathway to activate the innate immune system (389, 390). The Eimeria tenella derived protein, was isolated from bovine small intestinal extracts and was shown to have remarkable anti-cancer activity and be a potent stimulator of innate immune responses in various mouse models in vitro and in vivo (386, 391, 392). The rEA protein, we believe, might possess all the properties of an ideal immunologic adjuvant, as it can be fairly inexpensive to produce, is extremely stable and can be stored for long periods of time (over 24 month, data not shown) without losing activity. The rEA has been shown to be safe and very well-tolerated in human clinical trials (137). In mice, rEA augments activation of the innate immune system, presumably by activating PRRs (TLRs and/or possibly others) and thereby increasing adaptive immune responses to co-administered antigens (133, 139). It has been suggested that TLR11 is involved in rEA signaling (135), a notion that is primarily based on high 165 sequence homology (67%) between rEA and T. gondii profilin-like protein, the latter being the only confirmed ligand for TLR11 (138). In this study we confirmed that administration into mice, or direct exposure of immune cells to rEA protein results in (1) activation of important immune cell types (DCs, macrophages, NK, NKT, B and T cells), including (2) pro-inflammatory cytokines/chemokines release, both globally and specifically by DCs, and highly robust IFN production by NK cells, and (3) Erk1/2 phosphorylation. Therefore, we showed that rEA, similarly to other TLR-agonist-based adjuvants, activated an innate immune profile that resulted in robust activation of innate immune cells and induced multiple cytokines/chemokine pathways. Our results also confirmed that these responses are completely dependent upon MyD88, as genetic knockout of this adaptor protein results in complete ablation of these responses both in vitro and in vivo. When we similarly investigated the role of TRIF, the other major TLR adaptor protein, we encountered unexpected results. Specifically, TRIF-KO mice showed dramatic increases, in immune cell activation and other rEA triggered responses, when compared to rEA treated WT mice. Specifically, we found that IL6, IL12p40, IL2, IL1, IL1, and MIP1 production was induced by rEA treatment in TRIF-KO mice in vivo and in DCs derived from these mice in vitro to significantly higher levels as compared to similar assays performed in rEA-treated WT mice. Note, that rEA mediated induction of IL12p70, a cytokine abundantly produced by DCs that activates NK cells, was also found to be negatively regulated by TRIF after exposure to rEA (387, 393). Therefore, in response to rEA-mediated stimulation, TRIF acts as a suppressor of the rEA-induced (MyD88-dependent) activation of DCs, macrophages, NK, NKT, T, and B cells in vivo. Despite the lack of TRIF activity in MyD88/TRIF-DKO mice, rEA responses were still 166 ablated which indicates that in the absence of MyD88, TRIF cannot carry out its suppressive activity. Since the lack of TRIF protein does not rescue the phenotype in MyD88/TRIF-DKO mice, this confirms an essential role of MyD88 in mediating these responses and suggests that TRIF protein acts as a suppressor downstream of MyD88 and/or MyD88 and TRIF adaptor molecules orchestrating the induction of pro-inflammatory immune responses following rEA injection. Numerous studies have described important roles for TRIF as a TLR system adaptor protein that acts to enhance TLR-based signaling (predominantly when TLR3 and TLR4 ligands are interrogated, thereby promoting antimicrobial responses (385, 394, 395). Specifically, TRIFKO mice showed dramatically impaired lung clearance of Pseudomonas aeruginosa infections; a response that is correlated with blunted cytokine induction (e.g. RANTES, IL1, MIP2) and reduced NFB activation present in both alveolar and peritoneal macrophages from these mice (396). Lack of TRIF protein also results in reduced induction of antigen specific humoral and cellular immune responses in other models (397). Specifically, T cells derived from TRIF-KO mice had dramatically reduced IFN production and CXCR3 expression upon antigen/LPS treatment as compared to WT mice (398). In DCs, TRIF functionality was confirmed to be important for upregulation of CD40 and CD86 co-stimulatory molecules (399). Moreover, TRIF protein along with IPS1 (RIGI/Mda5 pathway protein) are key adaptors in mediating Poly-ICtriggered adjuvant effects (397). Conversely, there is very little data available that describes suppressive roles of TRIF protein on TLR/PRR signaling. Specifically, we have previously demonstrated that a lack of functional TRIF protein results in increased transgene (e.g. -Gal) specific IgG titers in mice injected with Ad5-LacZ, suggesting that TRIF may act as a negative regulator of Ad-mediated 167 antibody responses in mice (400). Other researchers have demonstrated that TRIF has an inhibitory role in TLR5-mediated responses through induction of TLR5 degradation (401). This phenomenon may not be limited to TLR5, as it has been suggested that TRIF can also induce degradation of other TLRs, including TLRs 3, 6, 7, 8, 9, and 10 (401). To determine if the suppressive role of TRIF in DCs is more global than previously considered, we have stimulated isolated DCs with TLR4 (LPS), TLR7/8 (R848) or TLR9 (ODN2006) agonists and found that the presence of the TRIF adaptor protein significantly suppresses release of pleiotropic proinflammatory cytokines by DCs in response to these agonists. These studies suggest that suppressive activities of TRIF, in regard to pathogen-induced innate immune responses, may be more prevalent than currently appreciated. Activation of pro-inflammatory cytokines/chemokines is a critical step during DC activation/maturation. It has been suggested that TRIF may be required for inducing immunological tolerance by augmenting IL10 production (402, 403). We found, however, that in rEA (or other TLR agonist) treated TRIF-KO mice, amounts of IL10 (both in plasma and in isolated DCs) were indistinguishable to identically (rEA) treated WT mice. To successfully bridge DC activation to induction of substantial adaptive immune responses (e.g. T cell activation), it is essential to have induction of co-stimulatory molecules such as CD40, CD80, and CD86 on the surface of DCs. In this study, we have shown that administration of rEA results in robust maturation of DCs, as evidenced by not only increased expression of co-stimulatory molecules on these DCs, but also that rEA increased cytokines and chemokine production. While these responses were completely abrogated in rEA-treated MyD88-KO mice, most of them were dramatically enhanced in TRIF-KO mice when compared to rEA treated WT mice. 168 From this information, we are proposing a model of rEA signaling in DCs. The rEA protein likely interacts with a PRR, (likely TLR11 in mice, unknown in humans (135)), that senses rEA. The MyD88 adaptor protein gets recruited which allows MAP kinases downstream of MyD88 to become activated (pErk1/2, p38). pErk1/2 is capable of activating various transcription factors (e.g. AP1) which further activates pro-inflammatory genes, including those of pro-inflammatory cytokines/chemokines (395, 398, 404, 405). In contrast, functional TRIF protein acts as a negative regulator of the rEA-induced signaling. As a result of TRIF’s inhibitory effects, DCs have a reduction in surface expression of maturation markers, as well mitigated release of pro-inflammatory cytokines/chemokines (387) (Figure 39). Figure 39: TRIF acts as a negative regulator of rEA-induced signaling and downstream responses in DCs: model of action. rEA is a protein derived from Eimeria tenella (133, 386) and is highly homologous to Toxoplasma gondii profilin-like protein (138). T. gondii has been shown to signal, at least in part, through TLR11; therefore, it is likely that TLR11 is one of the main pattern recognition receptors (PRRs) utilized by rEA. We have shown that rEA-triggered responses in vivo are completely dependent on MyD88. MAP kinases consequently become activated downstream of MyD88. Importantly, functional TRIF protein inhibited rEA-mediated signaling. DCs are a major cell type in mediating rEA responses and, under TRIF’s inhibitory effects, have mitigated induction of surface expression of maturation markers and stunted release of pro-inflammatory cytokines/chemokines. Release of these molecules is critical for rapid amplification of immune responses and is mediated by autocrine and paracrine signaling (387). We have confirmed that TRIF protein reduces release of pro-inflammatory cytokines/chemokines in response to rEA, resulting in reduced activation of NK, NKT, T, and B cells as well as reduced IFN production by NK cells. 169 Figure 39: TRIF acts as a negative regulator of rEA-induced signaling and downstream responses in DCs: model of action. 170 In conclusion, our murine models have shown that rEA activates multiple immune cell types, stimulates pro-inflammatory cytokine/chemokine release, and triggers the MAPK pathway in a MyD88 dependent manner. We have also confirmed that DCs are the main subset of innate immune cells that mediate rEA-triggered responses, thus justifying future studies on isolated DCs and the potential use of rEA adjuvant in a DC-vaccine setting (406). Importantly, our studies of rEA unveiled a suppressive activity to the TRIF adaptor protein. This clearly justifies future testing of specific TRIF inhibitors or knockdown models prior to rEA/antigen administration as a means to further enhance induction of antigen specific adaptive immune responses. Whether TRIF acts to negatively regulate other adjuvants or TLR-mediated activations is a question that will require future investigations. The latter, however, highlights the complexities of TLR adaptor functions, and should temper efforts that target these proteins with agonists or antagonists, as the end result of such interventions may run counter to hoped for outcomes, and could be detrimental in some situations (407) 171 Acknowledgements We wish to thank the Michigan State University Laboratory Animal support facility for their assistance in the humane care and maintenance of the animals utilized in this work. A. A. was supported by the MSU Foundation, as well the Osteopathic Heritage Foundation. YAA was supported by the King Abdullah bin Abdulaziz Scholarship, Ministry of Higher Education, Kingdom of Saudi Arabia. 172 Chapter V Preventing CRACC receptor upregulation in antigen presenting cells improves induction of antigen specific adaptive immune responses by vaccines . 173 5.1. Introduction The innate immune system relies heavily on a variety of transmembrane, intracellular, or secreted pattern-recognition receptors (PRRs), each of which are vital for recognition of specific molecular structures found on, or within potentially infectious agents, such as viruses or microbes (408). Activation of these PRRs triggers signaling pathways that regulate the transcription of pro-inflammatory cytokine and chemokines genes, as well as other innate immune defense responses, responses that also help shape subsequent, antigen specific adaptive immune responses, (5, 408). The innate immune system also has a key role in initiating and orchestrating the adaptive immune responses to antigens presented during vaccinations (108, 385). These facts suggest that specific modulation of innate immunity during vaccination may allow for the development of improved therapeutic and preventative vaccines (120). A highly characterized family of innate immune receptors, the Toll-like receptors (TLRs) , have been targeted for modulation in pre-clinical and clinical vaccine applications (4). Triggering TLR signaling by use of specific agonists or over-expressing TLR adaptors, (MyD88 or TRIF), has been shown to induce pro-inflammatory cytokine secretion and to augment the adaptive immune responses toward target antigens (133, 293, 409). Another important family of immunoreceptor that plays a critical role in immune regulation is the signaling lymphocytic activation molecule (SLAM) family of receptors (289). SLAM receptors function as adhesion molecules on the surface of many hematopoietic cells. They serve as costimulatory molecules that regulate intracellular signaling pathways that govern the function of T-, B-, NK-, macrophages, and dendritic cells (DCs) (410). The SLAM family of receptors currently comprise six distinct innate and adaptive immune-cell specific members, respectively named SLAM (CD150), 2B4 (CD244), Ly9, CD84, NTB-A (natural killer, T and B 174 cell antigen; Ly108 in the mouse) and CRACC (CD2-like receptor activating cytotoxic cells) (149, 287). All SLAM members except 2B4, (which interacts with CD48) are self-ligands. They initiate intra-cellular signaling via recruitment of specific adaptors (287). For example, activation of the CRACC receptor by CRACC-specific antibodies or self ligation to CRACC being expressed on a neighboring cell promotes NK cells cytotoxicity (150, 151, 411, 412). In addition, in CD40L-activated human DCs, antibody-mediated ligation of SLAM (CD150) augmented the secretion of pro-inflammatory cytokines IL-12 and IL-8 (291). Furthermore, the SLAM (CD150) receptor was also found to regulate the production of IL-6 and IL-12 by mouse peritoneal macrophages (292). The SLAM-associated protein family of adaptors includes three members named SAP, Ewing’s sarcoma-associated transcript-2 (EAT-2), and EAT-2-related transducer (ERT; ERT is however a non-functional pseudo-gene in humans). These adaptors associate with phosphorylated tyrosine-based motifs (‘immunoreceptor tyrosine based switch motifs’ (ITSMs)) in the cytoplasmic domains of SLAM family receptors with high affinity and specificity. All SLAM receptors can interact with either of the adaptors, except CRACC which interacts only with EAT-2 and not SAP (140, 150, 151). SLAM family of adaptors regulate SLAM induced intracellular signaling in a variety of immune cells (286). EAT-2 and ERT directly transduce SLAM initiated signals via phosphorylation of tyrosine residues located in their short carboxylterminal tails (165). In contrast, SAP regulates SLAM signaling by recruiting the protein tyrosine kinase FynT (413). Since EAT-2 is the only known SLAM-associated adaptor protein expressed in DCs and macrophages, it has been proposed that EAT-2 facilitates SLAM dependent pro-inflammatory cytokine expression in these cell types (290). In addition, SLAM receptor (CD150) has been 175 found to function as a critical microbial sensor that positively regulates bacterial killing by macrophages (414). In a recent report, it was also shown that the SLAM family member CRACC positively regulates NK cell function by a mechanism dependent on the adaptor EAT-2, but not the related adaptor SAP (151). Furthermore, the CRACC receptor can also have inhibitory functions in T cells during antigen presentation. Interestingly, T cells do not express the EAT-2 adaptor (151). We have previously described a novel immunostimulatory function for the SLAM family receptors adaptor EAT-2 in a vaccine model system in mice, a system in which over-expression of EAT-2 resulted in improved induction of robust antigen specific adaptive immune responses after Adenovirus (Ad) mediated transfer and expression of antigen encoding genes (415). In this study, we set out to investigate the mechanism of action of EAT-2. Specifically, we studied the impact of EAT-2 over-expression in DCs and macrophages function, to determine how EAT-2 functions to alter induction of antigen specific adaptive immune responses. The results of our experiments show that Ad mediated expression of EAT-2 activate signaling cascades that induce downstream activation of ERK dependent signaling pathways in DCs and macrophages. This activity correlated with suppressed expression of the CRACC receptor on DCs and macrophages, a response that is completely dependent upon the interaction between the EAT-2 adaptor SH-2 domain and the phosphorylated ITSMs of the SLAM receptors. Thus, EAT-2 functions are not only required to activate NK cell effector and regulatory functions, but, like other PRR adaptors, induces critical regulatory pathways crucial for the improved function of DCs and macrophages during antigen presentation to the adaptive immune system. 176 5.2. Results SLAM family receptors are self ligands (416) and homophilic interactions between these receptors triggers immune cell activation (417). Most SLAM family receptors are expressed on dendritic cells and macrophages (410). Therefore, we set out to evaluate the mechanisms underlying EAT-2 involvement in the process of antigen presentation in APCs. We initially investigated the impact that EAT-2 over-expression has on the expression levels of SLAM receptors in macrophages. To define the expression pattern of these receptors, RAW264.7 macrophages were mock infected, or infected with EAT-2 expressing Ads, or a control Ad vector (Ad-Null). Six hours post infection (hpi), the expression levels of the SLAM family receptors SLAM, 2B4, CRACC, CD84, and Ly-9 were analyzed by quantitative RT-PCR as previously described (101). We found that Ad vector infection itself significantly (p<0.001) induced the expression of the SLAM family member CRACC, but not other SLAM family members in macrophages (Figure 40A). In dramatic contrast, infection of macrophages with an EAT-2 expressing Ad significantly reduced Ad induction of CRACC receptor gene expression (p<0.001) (Figure 40A). We also attempted to evaluate the expression level of the SLAM family member Ly108, however its expression was not detectable in these cells, a result consistent with previously published studies (418). A time course study confirmed and extended these results. For example, at 3 hpi the expression level of the CRACC receptor was significantly and equivalently induced (p<0.01) by both the Ad-EAT2 and Ad-Null vectors (Figure 40B and supplementary Figure 40A). However, at later time points (6, 15, and 24 hpi), Ad infection continued to significantly (p<0.001) induce CRACC receptor expression, while Ad infection coupled with EAT2 overexpression significantly (p<0.001) reduced CRACC expression at these same time points (Figure 40B). 177 Figure 40: EAT-2 functions as a negative regulator of Adenovirus mediated induction of CRACC receptor expression on macrophages in vitro. RAW264.7 cells (300,000 cells/ well) were mock infected or infected with 20,000 vector particles/ cell of Ad-EAT2 (black) AdEAT2(R31Q) (dash line) or Ad-Null (gray). (A) Quantitative RT-PCR for SLAM family receptors six hours post infection (6 hpi). (B) Time course study for CRACC receptor expression in RAW264.7 macrophages. Statistical analysis was completed using One Way ANOVA with a student- Newman-Keuls post-hoc test, p<0. 05 was deemed a statistically significant difference. Data are representative of five independent experiments with similar results. Samples were plated in quadruplicate and are expressed as mean ± SD (* denotes p<0. 05, ** denotes P< 0.001 statistically different from mock infected cells). 178 Figure 40: EAT-2 functions as a negative regulator of Adenovirus mediated induction of CRACC receptor expression on macrophages in vitro. 179 By 15 hpi, both Ad-EAT2 and Ad-Null induced similar levels of ADAR, ICAM-1, NOD1, TNF-α, IL-6, and IL-15 genes, results that support the notion that EAT-2 over-expression specifically repressed only CRACC receptor RNA levels after Ad infection of macrophages (Figure 41). Figure 41: EAT-2 over-expression induces similar transcript levels of innate immune responses genes as compared to adenovirus control. RAW264.7 cells (300,000 cells/ well) were mock infected or infected with 20,000 vector particles/ cell of Ad-EAT2 (black) or Ad-Null (gray). Quantitative RT-PCR for ADAR, ICAM, and NOD-1, TNF-a, IL-6 and IL-15 gene expression in RAW264.7 macrophages at 15 hpi following Ads infection. Statistical analysis was completed using One Way ANOVA with a student- Newman-Keuls post-hoc test, p<0.05 was deemed a statistically significant difference. Data are representative of two independent experiments with similar results. Samples were plated in quadruplicate and are expressed as mean ± SD (* denotes p<0.01, *** denotes P< 0.001 statistically different from mock infected cells). 180 Figure 41: EAT-2 over-expression induces similar transcript levels of innate immune responses genes as compared to adenovirus control. 181 We also directly measured EAT-2 transcript levels at the time points tested. We observed minimal induction of EAT-2 (two fold over mock) following Ad-Null infection (Figure 42A), suggesting minor activation of EAT-2 signaling pathway following Ad infection. In contrast, significant increases in EAT-2 transcript were detected in Ad-EAT2 transduced cells, due to high level EAT-2 gene expression from the Ad vector (Figure 42B). Figure 42: EAT-2 transcript levels following Ad-EAT2 and Ad-EAT2 (R31Q) infection (A) EAT-2 transcript level at 6 and 15 hpi after Ad-Null infection. (B) Time course study for EAT-2 transcript level in RAW264.7 cells after Ad-EAT-2 infection. (C) EAT-2 transcript level at 15 hpi following Ad-EAT2 or Ad-EAT2 (R31Q) infection. Samples were plated in quadruplicate and are expressed as mean ± SD. 182 Figure 42: EAT-2 transcript levels following Ad-EAT2 and Ad-EAT2 (R31Q) infection. 183 To evaluate if protein levels of the CRACC receptor correlated with CRACC receptor RNA transcript levels, we utilized FACS analysis. RAW264.7 cells were infected with Ad-EAT2 or Ad-Null for 20 or 72 hpi. At 20 hpi, both Ad-EAT2 and Ad-Null significantly induced (p<0.001) the cell surface expression of CRACC receptor to similar levels, (data not shown). At 72 hpi, Adenovirus infection significantly (p<0.001) induced the cell surface expression of CRACC receptor (Figure 43A). Importantly, infection with Ad-EAT2 significantly (p<0.01) reduced the number of CRACC expressing RAW264.7 cells as well as the number of CRACC molecules per cell, as compared to Ad-Null infected cells (Figure 43A). In contrast, the cell surface expression of 2B4, CD84, and Ly-9 were similarly expressed in both Ad-EAT2 and AdNull infected cells (data not shown). We also utilized a more relevant in vitro primary macrophage cell culture system. Bone marrow cells harvested from C57BL/6 mice were differentiated into macrophages (BMDMs). BMDMs were infected with Ad-EAT-2 or Ad-Null for 72 hpi and the cell surface expression of CRACC receptor was evaluated by flow cytometry. Similar to RAW264.7 cells, Ad-Null infection resulted in significantly (p<0.001) increased expression of the CRACC receptor, while infection of BMDMS with Ad EAT-2 significantly (p<0.001) reduced the number of CRACC expressing cells we detected, as well as the number of CRACC molecules per cell detected by this method (Figure 43B). To confirm this mechanism is + not limited to one APC type, we isolated murine splenic CD11c DCs and evaluated CRACC receptor cell surface expression by flow cytometry. Similar to macrophages, Ad infection of + CD11c DCs significantly (p<0.001) increased the cell surface expression of the CRACC receptor (Figure 43C). Importantly Ad mediated transduction of EAT-2 significantly (p<0.05) reduced CRACC receptor expression in these same cells (Figure 43C). Thus, EAT2 over- 184 expression by Ads prevents Ad induced transcription of the CRACC receptor gene, resulting in reduced CRACC protein levels in Ad transduced macrophages and DCs in vitro. Figure 43: EAT-2 over-expression reduce protein level of CRACC receptor on DCs and macrophages in vitro. In vitro cultured (A) RAW264.7 macrophages, (B) murine bone marrow + derived macrophages (BMDMs), or (C) isolated murine splenic CD11c DCs, (300,000 cells/ well) were mock infected or infected with the Ad-EAT2 (black) or Ad-Null control (gray) for 72 hours at the multiplicity of infection (MOI) of 20,000 vector particles/ cell for RAW264.7 and + BMDMs or MOI of 5000 for CD11c DCs. At 72 hpi, cells were stained with APC-conjugated CRACC specific antibody and analyzed on an LSR-II flow cytometer. (A) Expression of CRACC receptor on RAW264.7 following infection with Ad-EAT2 or Ad-Null. (B) Expression + of CRACC receptor on BMDMs. (C) Expression of CRACC receptor on murine splenic CD11c cells. Statistical analysis was completed using One Way ANOVA with a student- NewmanKeuls post-hoc test, p<0.05 was deemed a statistically significant difference. Data are representative of three independent experiments with similar results. Samples were plated in quadruplicate and are expressed as mean ± SD (*, denotes p<0.05, ** denotes P<0.01, *** denotes P< 0.001 statistically different from mock infected cells). 185 Figure 43: EAT-2 over-expression reduce protein level of CRACC receptor on DCs and macrophages in vitro. 186 To investigate whether EAT-2 over-expression negatively regulates CRACC receptor expression in vivo, C57BL/6 mice were either mock injected (PBS), or intravenously injected 10 with 7.5 × 10 vps of Ad-EAT-2 or Ad-Null as previously described (103). At 10 hpi, Ad infection itself significantly (p<0.01) increased the expression level of CRACC receptor on + - CD11c CD11b DCs, as no significant differences were observed between Ad-EAT2 and AdNull infected mice at this time point (Figure 44A). Importantly, at 48 hpi, consistent with the in + - vitro results, the level of CRACC receptor expression on CD11c CD11b DCs was significantly reduced in Ad-EAT2 injected mice as compared to Ad-Null injected controls (Figure 44B). These results further confirm that administration of Ad vectors induces CRACC expression, but that EAT-2 over-expression prevents this induction, both in vitro and in vivo. Figure 44: EAT-2 over-expression negatively regulates CRACC expression in dendritic cells in vivo. C57BL/6 mice (n=4) were either mock injected, or intravenously injected with 10 7.5×10 vps of either Ad-EAT2 or Ad-Null vectors. Splenocytes were harvested at 10 hpi (A) + - or 48 hours (B) post-injection and CRACC receptor expressing CD11c CD11b DCs was identified by using a LSR-II flow cytometer. The bars represent mean ± SD. Statistical analysis was completed using One Way ANOVA with a Student-Newman-Keuls post-hoc test, p<0.05 was deemed a statistically significant difference. * denotes p<0. 05, ** denotes p<0.01 statistically different from mock injected animals. 187 Figure 44: EAT-2 over-expression negatively regulates CRACC expression in dendritic cells in vivo. 188 The tyrosine-based motif (ITSM) present in the cytoplasmic domain of SLAM family receptors interacts with the Src homology 2 (SH2) domains present in SAP adaptors (140, 152). To investigate whether this specific interaction was required for EAT-2 to prevent Ad induced expression of the CRACC receptor on APCs, we generated a mutant form of the EAT-2 adaptor, EAT-2(R31Q) that contains a missense mutation that replaces the positively charged arginine residue present at this location with a glutamine. Based upon studies in the highly homologous SAP adaptor, we hypothesized that the mutation would disrupt the putative phosphotyrosinebinding pocket of EAT-2 (419). Once generated by targeted mutagenesis, the Ad vector expressing EAT-2(R31Q), Ad-EAT2 (R31Q), was generated and successfully purified to high titer. We first confirmed that the Ad-EAT2 (R31Q) mutant virus expressed similar levels of the EAT-2 transcript per vector particle number as the Ad vector expressing the wild type version of EAT-2 (Figure 45A). We then evaluated CRACC receptor expression on RAW264.7 cells at 15 hpi following identical infection with Ad-EAT2, Ad-EAT2 (R31Q), or Ad-Null control. Consistent with the results obtained previously (see Figure 41A and B), infection with wild type (WT) EAT2 expressing Ads significantly (p<0.01) prevented Ad-mediated induction of CRACC receptor compared to Ad-Null infected cells (Figure 45B). Conversely, the inhibitory function of EAT-2 was completely eliminated when the identical experiment was performed utilizing the Ad expressing the EAT-2 phosphotyrosine binding pocket mutant (Figure 45B). Importantly, no significant differences in CRACC levels were observed between Ad-Null and Ad-EAT2 (R31Q) viruses (Figure 45B). We also evaluated the protein levels of CRACC at 72 hpi by flow cytometry. Consistent with the transcript level, infection with Ad-EAT2 (R31Q) virus resulted in enhanced protein levels of CRACC (p<0.001) as compared to Ads expressing the WT version of EAT-2 (Figure 45C). These results strongly suggest that CRACC receptor down-regulation by 189 EAT-2 is specifically regulated by direct interaction between the SH-2 domain of EAT-2 and the phosphorylated ITSMs of SLAM receptors present on APCs. Figure 45: Mutant form of EAT-2 adaptor does not prevent CRACC upregulation by Ads. RAW264.7 cells (300,000 cells/ well) were mock infected or infected with 20,000 vector particles/ cell of Ad-EAT2 (black) Ad-EAT2(R31Q) (dash line) or Ad-Null (gray). (A) EAT-2 transcript level at 15 hpi following Ad-EAT2 or Ad-EAT2 (R31Q) infection. (B) Quantitative RT-PCR for CRACC transcript at 15 hpi derived from Ad-EAT2, Ad-Null, or Ad-EAT2 (R31Q) infected RAW264.7 cells. (C) FACS analysis of CRACC receptor on RAW264.7 following infection with Ad-EAT2, Ad-EAT2 (R31Q) or Ad-Null. Statistical analysis was completed using One Way ANOVA with a student- Newman-Keuls post-hoc test, p<0.05 was deemed a statistically significant difference. Data are representative of three independent experiments with similar results. Samples were plated in quadruplicate and are expressed as mean ± SD (*, denotes p<0.05, ** denotes P<0.01, *** denotes P< 0.001 statistically different from mock infected cells). 190 Figure 45: Mutant form of EAT-2 adaptor does not prevent CRACC upregulation by Ads. 191 The signaling pathways that are activated by EAT-2 in APCs are not completely defined. Previous reports have shown that downstream of SLAM family receptors, EAT-2 activates the Src-kinase FynT (420), phospholipase C gamma (150, 167), and PI3K (150) signaling pathways in NK cells. We set out to investigate which signaling pathway(s) are activated by EAT-2 and are required for preventing Ad-mediated induction of CRACC receptor on APCs. For this, a variety of pharmacological inhibitors were used and the expression of CRACC receptor following Ad-EAT2 or Ad-Null infection was evaluated. RAW264.7 cells were pre-treated with pharmacological inhibitors that block the Src-kinase pathway (PP2), the PI3K pathway (wortmannin), the PLCγ pathway (U-73122), and the ERK MAP kinase pathway (PD98059) prior to Ad infection, and the expression of CRACC receptor was evaluated by quantitative RTPCR at 15 hpi. Inhibition of the Src-kinase or the PI3K signaling pathways did not affect the ability of EAT-2 to prevent Ad induced CRACC receptor expression (Figure 46A). However, blocking the ERK MAP Kinase signaling pathway by use of the MEK kinase inhibitor PD98059, or the PLCγ signaling pathway by the use of U-73122, completely eliminated the ability of AdEAT-2 to prevent Ad-mediated induction of CRACC receptor expression in RAW264.7 cells (Figure 46B). Figure 46: EAT-2 requires functional ERK and PLCγ pathways to down-regulate CRACC receptor on APCs. CRACC receptor expression in RAW264.7 cells at 15 hpi following AdEAT2 (black) or Ad-Null (gray) (MOI of 20,000) in the presence or absence of the following inhibitors: PD98059 (5 µM), U-73122 (1 µM), PP2 (1 µM), and Wortmannin (5 µM). (A) CRACC receptor expression in the presence or absence of Src-kinase or PI3K signaling pathways inhibitors. (B) CRACC receptor expression in the presence or absence of ERK or PLCγ signaling pathways inhibitors. Statistical analysis was completed using Two-Way 192 ANOVA with a Bonferroni post-hoc test p<0.05 was deemed a statistically significant difference. * denotes p<0.05, ** denotes p<0.001 statistically different from mock infected cells. # denotes p<0.01, ## denotes p<0.001, significantly different from Ad-infected kinase inhibitors non-treated cells. 193 Figure 46: EAT-2 requires functional ERK and PLCγ pathways to down-regulate CRACC receptor on APCs. 194 To further confirm the role of a functional ERK MAPK signaling pathway in EAT-2 mediated prevention of CRACC receptor up-regulation in Ad infected RAW264.7 cells, we examined ERK phosphorylation in Ad infected cells. Consistent with our previously published work (421), Ad infection of RAW264.7 cells induced ERK phosphorylation as early as 4 hpi. At 4, 8, and 12 hpi, no significant differences were observed between Ad-EAT2 and Ad-Null infected cells (Figure 47A). Importantly, at16 and 24 hpi, Ad-EAT-2 induced higher ERK phosphorylation compared to Ad-Null infected control (Figure 47A and B). Figure 47: EAT-2 over-expression induces ERK phosphorylation in RAW264.7 cells. Western blot analysis for ERK phosphorylation following Ad-EAT2 or Ad-Null infection at the indicated time points was performed. Phosphorylated Erk1/2 and Erk2 levels were determined as described in materials and methods using LI-COR Odyssey. To control for loading, quantification was performed after normalizing the pErk1/2 to Erk2 levels. A two-tailed homoscedastic Student’s t-test was used to calculate differences. p<0.05 was deemed a statistically significant difference. 195 Figure 47: EAT-2 over-expression induces ERK phosphorylation in RAW264.7 cells. 196 DCs and macrophages express most of the SLAM family receptors. (422) Since the SLAM (CD150) receptor is involved in the regulation of cytokine secretion by human and mouse macrophages and dendritic cells (292) and the expression of these mediators enhanced the adaptive immune responses to vaccine antigens, we sought to evaluate a possible role for EAT-2 over-expression to augment the secretion of cytokine/ chemokine directly from macrophages. ERK MAPK dependence of these responses was also investigated. RAW264.7 cells were infected with Ad-EAT2 or Ad-Null and the concentration of multiple cytokines and chemokines present in the overlying medium was determined. Utilizing this system and multiplicity of infection, minor inductions of some cytokines and chemokines were quantified following Ad infection (Figure 48). Interestingly, Ad-EAT-2 infection of these cells induced a more robust secretion of cytokines and chemokines as compared to Ad-null infected cells (Figure 48). Specifically, EAT-2 over-expression significantly (p<0.001) induced higher levels of G-CSF, MIP-1β, IL-13, Eotaxin, and TNFα, as compared to Ad-Null infected cells (Figure 48). RAW264.7 cells pre-treated with PD98059 prevented EAT2 over-expression from inducing the secretion of TNF-α, G-CSF, and IL-6 (p<0.001) (Figure 48A). Conversely, PD98059 pre-treated RAW264.7 cells had an enhanced secretion of MIP-1β and IFNγ when infected with Ad-EAT2 as compared to infection with the Ad-null control virus (Figure 48B). In addition, RAW264.7 cells pre-treated with PD98059 partially prevented EAT2 over-expression from inducing the secretion of IL-1α, IL-1β, IL-2, IL-3, IL-4, IL-5, IL-9, IL-10, IL-12p40, IL-12p70, IL-13, IL-17, Eotaxin, KC, and GM-CSF (Figure 48C). Figure 48: EAT-2 is a critical cytokine and chemokines regulator in macrophages. RAW264.7 cells were mock infected or infected with Ad-EAT2 or Ad-Null for 72 hours at MOI of 20,000 in the presence or absence of ERK inhibitor (PD98059). Culture media was collected 197 at 72 hours post-Ad infection and was analyzed for cytokines and chemokines levels using a multiplexed bead array based quantitative system. Statistical analysis was completed using a one-way ANOVA with a Student-Newman-Keuls post-hoc test. Samples were plated in quadruplicate and are expressed as mean ± SD. *, *** -Indicate cytokine values that are statistically different from those in mock infected cells, p<0.05, p<0.001 respectively. #, ##, ###, indicate cytokine values that are statistically different from those in Ad-infected PD98059 nontreated cells, p<0.05, p<0.01, p<0.001 respectively. 198 Figure 48: EAT-2 is a critical cytokine and chemokines regulator in macrophages. 199 5.3. Discussion The development of advanced generation vaccines will be required to eradicate those pathogens still plaguing mankind. To develop these vaccines to their fullest potential will require in depth understanding of the ability of the innate immune system to facilitate induction of robust, antigen specific adaptive immune responses. Numerous systems are being evaluated in this regard, and each has benefits or limitations that will need to be fully ascertained before progress will be made in the elimination of diseases such as HIV-AIDS, malaria, and tuberculosis. We have investigated several vaccine platforms, and discovered that Ad mediated transduction of the SLAM family of receptors adaptor EAT-2 allowed for improved induction of antigen specific adaptive immune responses to Ad expressed antigens (103). In an effort to further understand the molecular mechanisms underlying this important discovery, we have shown here that use of Ad vectors in general results in the upregulation of the SLAM family member CRACC, a response that may limit the full potential of Ad vectors to induce maximal, antigen specific adaptive immune responses. This insight is provided by our results demonstrating that EAT-2 over-expression during Ad mediated antigenic gene transfer specifically prevents Ad induction of CRACC. This ability is at least dependent upon a functional ERK MAPK pathway, as well the potential interaction between the EAT-2 SH-2 domain and the phosphorylated ITSMs of SLAM receptors. The phenomenon of reduced CRACC receptor expression on APCs has been associated recently with increased antigeninduced CD4+T cells proliferation, as well, IL-2 and IFNγ, production, consistent with our recently published work (151, 191). 200 The SLAM family of receptors is an emerging family of immune receptors that regulate the functions of both innate and adaptive immune cells (410). CRACC is a novel member of the SLAM family (423, 424) and is expressed on human plasma cells, NK cells, activated CD8+ T cells, activated B cells, macrophages, and dendritic cells (422, 424). Our results demonstrate that in vivo, Ad-EAT-2 transduced APCs function as a more potent APC as compared to conventional Ad transduced APCs, and this activity is correlated with a reduced abundance of cell surface CRACC receptor. This phenomenon may diminish CRACC-CRACC interactions at the immunological synapse between Ad-EAT2 transduced DCs and potentially responsive naïve T cells, and based upon these and our previous results, results in improved induction of antigen specific adaptive immune responses (103). Decreased CRACC-CRACC interactions between EAT-2 over expressing APCs and naïve T cells may limit CRACC activation and phosphorylation within the T cells, preventing binding of inhibitory phosphatases to the phosphorylated ITSMs of the T cell CRACC receptor, and thereby enhancing the induction of robust antigen specific, T cell based adaptive immune responses. Whether or not other vaccine platforms also increase CRACC expression is a question that our studies suggests should be addressed, as inhibition of this response may also facilitate improved effectiveness of those platforms as well. Alternatively (though not mutually exclusive), increased CRACC receptor expression on stressed cells has been associated with enhanced targeting of the cells for destruction by NK cells (150, 151). Cells transduced by first-generation Ad vectors are known to be targeted for elimination by NK cells (193, 425). Thus, decreased abundance of cell surface expression of CRACC by Ad-EAT2 transduced dendritic cells and macrophages may make them less 201 susceptible to CRACC-mediated NK cell mediated cytotoxicity, allowing for an improved potential to induce antigen specific adaptive immune responses. Previous reports have shown that the SLAM (CD150) receptor is involved in the regulation of cytokine secretion by human and mouse macrophages and DCs (292). Our findings here have complemented that work by demonstrating involvement of the SLAM family receptors adaptor EAT-2 in the secretion of multiple cytokine and chemokines, thereby linking SLAM signaling by EAT-2 to the mediation of cytokines and chemokines production in macrophages. Furthermore, a recent report demonstrated that activation of the SLAM family member CD84 enhanced the secretion of TNFα and MCP-1 by mouse RAW264.7 and bone marrow derived macrophages following TLR4 activation (426). This may suggest that the enhanced cytokine and chemokines profile after Ad-EAT-2 infection could be as a result of synergy between SLAM receptors signaling by EAT-2 and activation of TLR4, or other TLRs, signaling by Ads (8, 184). The enhanced production of these cytokines could also act as autocrine growth and differentiation factors as well as positive modulators of the immune responses by both DCs and macrophages. Further, autocrine stimulation by TNFα regulates the expression of many proinflammatory response genes, as well prime macrophages for enhanced responsiveness to cytokines and inflammatory stimuli (427). Production of TNFα after Ad-EAT2 infection was several orders of magnitude higher than its production after Ad infection. Thus, the present evidence supports the additional involvement of TNFα in Ad-EAT2 mediated–increases in cytokine expression. It is possible that other highly induced cytokines, such as G-CSF, IL-1β, and MIP-1β, also play a role in EAT-2 function in macrophages as well. Previous reports have shown that activation of CRACC recruits the EAT-2 adaptor, resulting in activation of the phospholipase Cγ signaling pathway in NK cells (150). Our results 202 suggested that the negative regulation of the CRACC receptor and the induction of several cytokines and chemokines by APCs after Ad mediated overexpression of EAT-2 are also mediated by a mechanism(s) that requires functional PLC-γ and ERK MAP kinase signaling pathways. Finally, since EAT-2, but not SAP binds to the CRACC receptor (150, 151), the reduced cell surface expression of CRACC in Ad-EAT2 transduced APCs may result in enhanced triggering of a negative feedback mechanism regulating CRACC receptor overall. These results, combined with our previous work (103, 191, 356), allows us to hypothesize a model for EAT-2 molecular mechanism in APCs (Figure 49). In this model, we hypothesize that upon EAT-2 binding to the phosphorylated ITSMs of SLAM family receptors results in recruitment of effector molecules in the phosphorylated C-tail of EAT-2 and the downstream activation of PLC-γ and the ERK signaling pathways, enhanced transcription of various cytokines and chemokines genes, as well as down-regulation of the CRACC receptor from the APC surface. Furthermore, augmenting SLAM signaling by EAT-2 induces higher levels of CD80 and CD86 co-stimulatory molecules, CD40, and MHC-II molecules on the APC surface that enhances transgene specific T cells proliferation and IFN-γ and IL-2 production (191, 356, 428). Figure 49: Model of EAT-2 molecular mechanism in APCs. In presence of high abundance of EAT-2 adaptor, EAT-2 binds to the phosphorylated ITSMs of SLAM family receptors and as a result the tyrosine residues at the EAT-2 C-terminal tail get phosphorylated. The phosphorylated tyrosine on EAT-2 C-tail serves as a docking site for down-stream, yet un-identified, mediators that transmit positive signals that result in down-stream activation of PLC-γ and ERK signaling pathways, enhanced transcription of various cytokines and chemokines genes, as well as downregulation of the CRACC receptor from the APC surface. Furthermore, augmenting SLAM 203 signaling by EAT-2 induces higher levels of CD80 and CD86 co-stimulatory molecules, CD40, and MHC-II molecules on the APC surface that enhances transgene specific T cells proliferation and IFN-γ and IL-2 production. In T cells, activation of CRACC receptor enhances the recruitment of inhibitory phosphatases that attenuate TCR-mediated activation. 204 Figure 49: Model of EAT-2 molecular mechanism in APCs. 205 In summary, we have determined that Ad vector mediated transduction of antigen expressing transgenes results in upregulation of CRACC on the surface of APCs. This effect inhibits the induction of maximal antigen specific adaptive immune responses by the Ad vector platform. Simultaneous overexpression of EAT-2 by Ad vectors prevents CRACC receptor upregulation on dendritic cells and macrophages improving the induction of proinflammatory cytokine and chemokine responses, maturation of APCs, and the induction of improved, antigen specific adaptive immune responses by Ad based vaccines. Future studies are needed to determine if other vaccine platforms can be similarly improved by modifying SLAM receptorSAP adaptor interactions. Acknowledgements: We wish to thank the Michigan State University Laboratory Animal support facility for their assistance in the humane care and maintenance of the animals utilized in this work. A.A. was supported by the National Institutes of Health grants RO1DK-069884, P01 CA078673, the MSU Foundation as well the Osteopathic Heritage Foundation. YAA was supported by the King Abdullah bin Abdulaziz Scholarship, Ministry of Higher Education, Kingdom of Saudi Arabia. Author Disclosure Statement: No competing financial interests exist. 206 Chapter VI Materials and Methods 207 6.1. Adenovirus vector construction All novel Ad vectors were constructed utilizing pAdEasy based system (429) with modifications. A first-generation, human Adenovirus type 5 derived replication deficient vector (deleted for the E1 and E3 genes) encoding EAT-2, HIV/Gag, CSP, rEA, and GFP as a transgene were used in these studies. Infectious titers of all Ads were determined by standard Tissue Culture Infectious Dose 50 (TCID50) method (AdEasy Adenoviral vector system manual, Qbiogene, Carlsbad, CA). Infections titer was calculated by using KARBER statistical method: 1 + d(S-0.5) TCID50/ml titer = 10 × 10 , where d is the log (10) of the dilution and S is the sum of ratios from the first dilution. All viruses were found to be RCA free both by RCA PCR (E1 region amplification) and direct sequencing methods. Ad vectors have also been tested for the presence of bacterial endotoxin as previously described (430) and were found to contain <0.01 EU per injection dose. 6.1.1. EAT-2 expressing Ads construction: The Open Reading Frame of EAT-2 gene (Genbank Accession #NM_012009), http://www.ncbi.nlm.nih.gov/nuccore/148747581, was excised using primers flanked by XhoI and XbaI restriction endonucleases (NEB, Ipswich, MA) from a plasmid (Open Biosystem, Huntsville, USA) and subcloned into the pShuttle vector which contains a CMV expression cassette. The resulting pAdTrack-EAT-2 shuttle plasmid was linearized with PmeI restriction enzyme and homologously recombined with the pAdEasyI Ad5 vector genome as previously described (429) yielding pAd-EAT2. HEK293 cells were transfected with PacI linearized plasmid and viable virus was obtained and amplified after several rounds of expanding infection. Ad-EAT2 virus was purified using a CsCl2 gradient as previously described (431). The titer 208 obtained was approximately 2.3 × 10 12 vp/ml. direct sequencing and restriction enzyme mapping were carried out to confirm the integrity of the EAT-2 sequence. 6.1.2. Ad-HIV/Gag construction: To construct Ad-HIV/Gag, the HXB2 Gag gene (Genbank Accession #K03455), http://www.ncbi.nlm.nih.gov/nuccore/1906382, was blunt end sub-cloned into the EcoRV site of pShuttle-CMV. Restriction digests and sequencing were used to confirm the sequence integrity and correct orientation of the resulting shuttle (pShuttle-CMV Gag). Recombination and viral propagation was completed as described above. 6.1.3. Ad-CSP construction: The Open Reading Frame (ORF) of the P. falciparum CS protein gene, composed of a codon optimized consensus of several P. falciparum CS protein sequences (Figure 16), was incorporated into plasmid pGA4 (GENEART, Burlingame, CA) and excised from pGA4 using endonuclease NheI (NEB, Ipswich, MA). The excised portion was subcloned into the pAd Shuttle vector containing a CMV expression cassette. Recombination and viral propagation was completed as described above. Direct sequencing and restriction enzyme mapping were carried out to confirm the fidelity of the CS protein sequence. 6.1.4. Ad-GFP and Ad-GFP/rEA construction: The Open Reading Frame of rEA gene was inserted into an identical CMV driven cassette directly upstream of the GFP cassette. The resulting pAdTrack-rEA shuttle with the pAdEasyI Ad5 vector genome was prepared and constructed as described above yielding AdGFP/rEA. Direct sequencing and restriction enzyme mapping were carried out to confirm the integrity of the rEA sequence. A direct comparison of transduction efficiency was completed for 209 Ad-GFP and Ad-GFP/rEA virus preparations using flow cytometry. Similar transduction efficiencies were observed. 6.2. Validation of viral particles (VP) titers of Ads 6.2.1. Silver Staining To verify that particle number quantification was accurate across all Ads constructed, 10 10 vps of lysed purified virions of each Ad were separated by 10% SDS-PAGE and subsequently stained with silver nitrate utilizing a Silver stain kit for proteins (Sigma, St. Louis, MO). The amount of hexon protein was quantified for each Ad vector by scanning densitometry using ImageJ software, ver. 1.29 (developed at the U.S. National Institutes of Health and available on the Internet at http://rsb.info.nih.gov/nih-image/). Results from this analysis indicated that the VP titers of all viruses determined by spectrophotometry fall within ~1.1 fold of each other. 6.2.2. Western Blotting To further verify that particle number quantification was accurate across all Ads constructed, 1010 of lysed purified virions of each Ad were separated by 10% SDS-PAGE and Western blotting was performed utilizing hexon specific antibodies (Abcam, Cambridge, MA). Electrophoretically separated capsid protein samples were transferred onto nitrocellulose membranes and probed with rabbit polyclonal Ad5 hexon specific antibody, followed by probing with a fluorescent secondary antibody as previously described (184). Membranes were scanned and hexon concentrations quantified using Licor’s Odyssey scanner (184). Results from this analysis indicated that the VP titers determined by spectrophotometry fall within ~1.3 fold of each other based on this assay, thus Ad5 vector preparations did not contain less virions as compared to conventional first generation Ad5 vectors based on this assay (data not shown). 210 6.3. Animal procedures All animal procedures were reviewed and approved by the Michigan State University ORCBS and IACUC. Care for mice was provided in accordance with PHS and AAALAC standards (ID number: A3955-01). Adult male C57BL/6 and Balb/c mice were purchased from The Jackson Laboratory (Bar Harbor, ME). Intravenous injection of animals (8-10 weeks old) consisted of injection (via the retro-orbital sinus) of 200µl of a phosphate-buffered saline solution (PBS, pH 7. 4), containing 7.5 × 1010 total virus particles after performing proper anesthesia with isofluorane. Plasma and tissue samples were obtained and processed at the indicated times post-injection as previously described (421). Intramuscular injections were completed by injection of the indicated virus particles in a total volume of 20 µl into the tibialis anterior of the right hind limb. All procedures with rAds were performed under BSL-2, and all vector treated animals were maintained in ABSL-2 condition. Care for mice was provided in accordance with PHS and AAALAC standards. Three groups of mice were analyzed in Ad-EAT2 and Ad-HIV/Gag co-injection study: C57BL/6 or BALB/cJ mice were mock-injected with PBS, control group injected with Ad5HIV/Gag +Ad-GFP, and the experimental group injected with Ad5-HIV/Gag+ Ad-EAT2. Control and experimental mice were sacrificed at different times after mock or virus treatment. Three groups of mice were analyzed in Ad-EAT2 or Ad-GFP/rEA and Ad-CSP coinjection study: BALB/cJ mice were mock-injected with PBS, control group injected with Ad5CSP +Ad-GFP, and the experimental groups injected with Ad5-CSP+ Ad-GFP/rEA or Ad5-CSP +Ad-EAT2. Control and experimental mice were sacrificed at different times after mock or virus treatment. 211 Four groups of mice were analyzed for rEA molecular mechanism study. MyD88-KO and TRIF-KO mice were kindly provided by Dr. Shizuo Akira. MyD88/TRIF-DKO mice were bred at Michigan State University. Intraperitoneal (IP) injection of animals (2-4 months in age) consisted of 100µl phosphate-buffered saline solution (PBS, pH 7.4) containing 100 ng rEA protein from Eimeria tenella as previously described (133, 386). rEA protein purification was performed as previously described (131) with minor modifications as described (131, 133). Plasma and tissue samples were obtained and processed at the indicated times post-injection as previously described (133). Importantly, IP route of rEA administration was confirmed to be more efficient that other widely used routes for adjuvant injection, such as intranasal or subcutaneous. To study rEA-triggered activation of immune cells by flow cytometry and bead array methods, plasma and spleen tissues were harvested at 6 hours post injection (hpi), whereas for studies measuring activation of signaling pathways the spleen and liver tissues were harvested at 0, 20, 40, 60, 90, and 120 minutes post-rEA injection. 6.4. Cytokine and chemokine analysis Proinflammatory cytokines/chemokines in the murine plasma collected in vivo or in the media collected from cultured DCs or RAW264.7 cells were measured utilizing a 7- or 23-plex multiplex based array system. Plasma samples were collected using heparinized capillary tubes and EDTA coated microvettes (Sarstedt, Nümbrecht, Germany) and centrifuged at 3400 rpm for 10 min to retrieve plasma samples. All procedures were performed exactly as previously described per the manufacturer’s instructions (Bio-Rad, Hercules, CA) via Luminex 100 technology (Luminex, Austin, TX) as previously described (133). 6.5. Quantitative RT-PCR Analysis 212 To determine relative levels of a specific, liver or spleen derived RNA transcript, corresponding tissues were snap frozen in liquid nitrogen and RNA was harvested from ≈100mg of frozen tissue using TRIzol reagent (Invitrogen, Carlsbad, CA) per the manufacturer’s protocol. Following RNA isolation, reverse transcription was performed on 180ng of total RNA using SuperScript II (Invitrogen, Carlsbad, CA) reverse transcriptase and random hexamers (Applied Biosystems, Foster City, CA) per manufacturer’s protocol. RT reactions were diluted to a total volume of 60µl and 2µl was used as the template in the subsequent PCR reactions. Primers were designed using Primer Bank web based software (http://pga.mgh.harvard.edu/primerbank/). Complete list of primers utilized in this study is available in table 3. Quantitative PCR (qPCR) was carried out on an ABI 7900HT Fast Real-Time PCR System using SYBR Green PCR Mastermix (Applied Biosystems) in a 15 µl reaction. PCRs were subjected to the following procedure: 95.0 1C° for 10 min followed by 40 cycles of 95.0 1C° for 15 s followed by 60.0 1C° for 1 min. The comparative Ct method was used to determine relative gene expression using GAPDH to standardize expression levels across all samples. Relative expression changes were calculated based on comparing experimental levels of a respective spleen transcript to those quantified in spleen samples derived from mock injected animals. 213 Table 3: List of primers, utilized in qRT-PCR experiments. A pair of Forward (For) and Reverse (Rev) primers is provided for every transcript tested by qRT-PCR based methods. The primers were designed as described above; the length of resulted PCR products was 100-160 nucleotides. Gene Forward primer (5' -> 3') Reverse primer (5' -> 3') EAT-2 CTGGGACTGATCTCAGGGTG GAAGGGAACGGGAGAATGGG CRACC CCGACTTGTGCCCTCACTTAG GAGCTGGGACTCTTTACCACT 2B4 CTCGGGGCCATCATTTGTTTC GCTAGAAGGGAGCTGAACATCA SLAM AAATCAGGGGTACGTTCTATGC TCCTGTGCGAAATATGACAGAC Ly108 TCTCCAGGGAACACTGTGTATG GGTTGGTTATAGCCGGTTAAAGC Ly9 TCAGGGATGCTAGGGGGTTC TTCGCTGACTTTGAGTCTGCC CD84 TTATTCTCATTCCGATGTTGGCA GTGGGTTGAGCATTTCTTGAAAC NOD-1 CCCCTTCCCAGCTCATTCG TGTGTCCATATAGGTCTCCTCCA TNFa CCCTCACACTCAGATCATCTTCT GCTACGACGTGGGCTACAG ADAR AGGATTGGTGAGCTCGTCAG GCCCTGTTTCTTGCTGTGTG ICAM-1 GGCATTGTTCTCTAATGTCTCCG GCTCCAGGTATATCCGAGCTTC IL-6 TAGTCCTTCCTACCCCAATTTCC TTGGTCCTTAGCCACTCCTTC IL-15 TCTCCCTAAAACAGAGGCCAA TGCAACTGGGATGAAAGTCAC 6.6 Isolation of Splenocytes Splenocytes from individual mice were harvested and processed by physically disrupting and facilitating passage of splenic tissue through a 40 µm sieve, followed by induction of RBC lysis by using 2 ml of ACK lysis buffer (Invitrogen, Carlsbad, CA) per homogenized spleen. 214 Splenocytes were subsequently washed two times with RPMI medium 1640 (Invitrogen, Carlsbad, CA) supplemented with 10% FBS, 2 mM L-glutamine, and 1% PSF (penicillin, streptomycin, fungizone), then resuspended and counted using the automated cell counter “countess” (Invitrogen). 6.7. Cell staining and flow cytometry Splenocyte preparations were evaluated for the presence of DC, macrophages, NK, NKT, 6 T, and B cell activation/maturation markers as previously described (172). 2×10 cells were stained with combinations of the following antibodies: APC-CD3, PerCpCy5.5-CD19, PE-Cy7NK1.1, and PE-CD69 (all 4 µg/ml) or PECy7-Cd11c, APCCy7-CD11b, APC-CD80, Pacific Blue-CD86, Alexa Fluor700-MHCII, FITC-CD40. For DCs and macrophages analysis, a dump channel had been applied using PerCpCy5.5-CD3/CD19/NK1.1 (all 4 µg/ml) antibodies (BD Biosciences, San Diego, CA). Cells were incubated on ice with the appropriate antibodies in 2.4G2 hybridoma cell supernatant for 30 minutes, then washed and sorted using a BD LSR II 6 instrument. For NK/NKT cells IFNγ intracellular staining, 3×10 splenocytes were incubated with Brefeldin A (1 µg/ml) in DMEM with 10%FBS/1xPSF for 4 hours at 37 ° C. Following incubation, splenocytes were washed two times with FACS buffer, incubated for 15 minutes with purified rat anti-mouse CD16/CD32 Fcγ block (BD Biosciences, San Diego, CA), surface stained with CD3-APC and NK1.1-PECy7 (8 µg/ml) for 30 minutes at 4 C, washed with FACS buffer, fixed with 2% formaldehyde (Polysciences, Warrington, PA) for 20 minutes on ice, permeabilized with 0.5% Saponin (Sigma-Aldrich, St. Louis, MO) for 20 minutes at room temperature, and incubated on ice with IFNγ-FITC (8 µg/ml) for 2 hours. Samples were analyzed on a BD LSR II instrument using FlowJo software (Tree Star, San Carlos, CA, USA). For 215 Adenovirus cell transduction, splenocytes were subjected to flow cytometry and the percentages of Ad-GFP transduced cells (FITC+) were calculated using FlowJo software. For BMDMs and RAW264.7 cells studies, the following antibodies were used: APCCD80, V450-CD86 (BD Biosciences, San Diego, CA), Alexa Floure700-MHC-II, and FITCCD40 (eBioscience, San Diego, CA) and CRACC-APC (cat# FAB4628A) (R& D System). For 7-color intracellular cytokines staining, cells were surface stained, fixed with 2% formaldehyde (Polysciences, Warrington, PA), permeabilized with 0.2 % Saponin (SigmaAldrich, St. Louis, MO), and stained for intracellular cytokines. Violet fluorescent reactive dye (ViViD, Invitorgen) was included as a viability marker to exclude dead cells from the analysis. For tetramer staining, blood was isolated by retro-orbital bleeds and PBMCs were isolated using Lympholyte-Mammal (Cedarlane, Burlington NC). Tetramer staining of PBMCs was completed using a PE conjugated MHC-I tetramer folded with the AMQMLKETI (HIV/Gag immunodominant epitope) or NYDNAGTNL (amino acids 43-51 of the CS protein sequence) peptide generated at the NIH Tetramer Core Facility. 6.8. In vitro cell culture: Bone marrow cells were extracted from the femurs and tibiae of male 6-8 weeks old Balb/c mice, and red cells were removed using ACK lysis buffer (Invitrogen, Carlsbad, CA). Bone marrow cells were cultured in Dulbecco modified Eagle medium (DMEM) supplemented with 10% fetal bovine serum (FBS) and 30% supernatant derived from confluent L929 cell cultures. At day 7, immature macrophages were collected and plated into 12-well plates for 24 hours. This procedure yields a pure population of macrophage colony-stimulating factordependent, adherent macrophages. Murine RAW264.7 macrophages (ATCC TIB71) were maintained in DMEM supplemented with 10% fetal bovine serum (FBS) and penicillin– 216 5 streptomycin following standard procedures. Suspensions of 5 ×10 cells were seeded into each well of 12-well plates. Then, the cells were incubated with 1 ml culture medium alone, or with medium containing Ad-EAT2, Ad-Null or Ad-GFP at the indicated MOIs and time points. For Pharmacological Inhibitors treatments, the following inhibitors were used: PP2 (1 µM), wortmannin (5 µM), PD-98059 (5 µM), and U-73122 (1µM) (all from BIOMOL). 6.9. Murine CD11c+ DCs isolation + Mouse DCs (CD11c splenic cells) were isolated from Balb/c or C57BL/6 mice using the magnetic assisted cell separation (MACS) system purchased from Miltenyi Biotech (Auburn, CA) utilizing the established protocols from the manufacturer. DC sorting by flow cytometry resulted in >95% pure CD11c positive cell population. The viability of recovered DCs was ~95% + as measured by trypan blue viability staining. Isolated CD11c cells were seeded at a density of 5 0.3×10 cells per well into 24-well plates in 300 µl/well complete medium (DMEM/F12 supplemented with 10% FCS and gentamicin (10µg/ml). Mouse DCs were infected with AdEAT2 or Ad-Null (MOIs of 5000) for 72 hpi at 37 C in 5% CO2 and 95% ambient air. Following incubation, cells were harvested and the expression of CRACC receptors was evaluated by flow cytometry. 6.10. Murine IL12p70 measurement by ELISA from isolated CD11c+ DCs + For mIL12p70 production from isolated CD11c DCs, an in vitro bioassay originally developed to monitor rEA activity during its purification and production, as well as provide + some insight into the mechanism of action of rEA, was previously described (131). CD11c DCs were isolated from C57BL/6 WT, MyD88-KO, TRIF-KO, or MyD88/TRIF-DKO mice. Cells 217 5 were seeded at a density of 0.3×10 cells per well into 96-well plates in 200 µl/well complete medium. The complete medium consisted of DMEM/F12 supplemented with 10% FCS, gentamicin (10 µg/ml), recombinant mouse GMCSF (1 ng/ml), recombinant mouse IL4 (1 ng/ml), recombinant mouse IFN (3 ng/ml), and an agonistic anti mouse CD40 antibody (0.5 mg/ml) (R&D Systems, Minneapolis, MN). This synergistic combination of cytokines has no significant effect on mouse IL12 release, but dramatically enhances the inducing effects of rEA -6 1 on mouse IL12 release (131). Mouse DCs were stimulated with rEA (10 -10 ng/ml) overnight (~18 hours) at 37 C in 5% CO2 and 95% ambient air. To study the specificity of TRIF inhibitory effects in DCs, various toll-like receptor agonists were added to mouse DCs, using the following concentrations: rEA (Barros Research Institute, Holt, MI, 100 ng/ml); E. coli 0111.B4 LPS (20 g/ml); R848 (0.5 g/ml) and ODN2006 (2.5 M). LPS, R848 and ODN2006 (TLR4, TLR7/8 and TLR9 agonists respectively) were purchased from InvivoGen, San Diego, CA, reconstituted with endotoxin-free water and diluted in culture media (DMEM/F12 + 10% FCS). Following incubation, culture medium was analyzed for mouse IL12p70 levels using an ELISA kit and following its enclosed instructions (R&D Systems, Minneapolis, MN) or for 23 mouse cytokines/chemokines using multiplex system (Bio-Rad, Hercules, CA). 6.11. CD8+ T cells depletion Analysis + CD8 T cells were depleted from pooled splenocytes preparations using MACS beads and LS columns per the manufacturer’s protocol (Miltenyi Biotec, Bergisch Gladbach, + Germany). % CD8- SFC = SFC CD8-dep/SFC CD8 ). Depletion was verified using FACS analysis using APC-CD8a, and Pacific Blue-CD4 antibodies (BD Biosciences, San Diego, CA). 6.12. ELISPOT Analysis 218 ELISpots were performed in accordance to manufacturer’s protocol using the Ready-set Go IFNγ or IL2 mouse ELISpot kit produced by eBiosciences (San Diego, CA). Splenocytes were stimulated ex vivo with 4μg/mL of the >98% pure CS protein immunodominant peptide NYDNAGTNL (amino acids 43-51 of the CS protein sequence) or HIV/Gag immunodominant peptide AMQMLKETI peptide (GenScript Piscataway, NJ). A library of 15mers overlapping by 5 amino acids spanning the entire CS or proteins non-repeating region or HIV/Gag protein was constructed and also used to stimulate splenocytes ex vivo (Biosynthesis Inc., Lewisville, TX). Spots were counted and photographed by an automated ELISPOT reader system (Cellular Technology, Cleveland, OH). Ready-set Go IFNγ and IL-2 mouse ELISPOT kits purchased from eBioscience (San Diego, CA). 6.13. In vivo CTL Assay For Ad-EAT2 and Ad-HIV/Gag co-injection study, Balb/c or C57Bl/6 mice were covaccinated with equivalent doses of Ad-HIV/Gag with either Ad-GFP or Ad-EAT2 (totaling 1× 7 9 10 vps for Balb/c and 1X 10 vps for C57Bl/6 mice). At 14 days, syngeneic splenocytes were isolated and either pulsed with an irrelevant peptide specific to the Plasmodium falciparum circumsporozoite antigen (NYDNAGTNL) or with the HIV-Gag immunodominant AMQMLKETI peptide or QBI# 304796 for 1 hour at 37ºC. Irrelevant peptide pulsed cells were subsequently stained with 1μM CFSE (CFSE with 10μM CFSE (CFSE amount of both CFSE High Low Low ) while Gag-peptides pulsed cells were stained ). Naïve and immunized mice were injected with equivalent and CFSE High stained cells via the retro-orbital sinus. After 5 hours, mice were terminally sacrificed and splenocytes were recovered and sorted on an LSRII flow cytometer. FlowJo software was used to determine percentages of CFSE stained cells. % 219 Specific killing = 1-((% CFSE High / % CFSE Low ) immunized / (% CFSE High / % CFSE Low ) non-immunized). For Ad-EAT2 and Ad-CSP co-injection study, same procedures were performed on Balb/c mice except that the in vivo CTL killing was evaluated 18 hours following the adoptive transfer of NYD- or non-specific peptide (AMQ)-loaded splenocytes injection. 6.14. Detection of CSP antibody in murine serum by ELISA ELISA-based antibody assays were completed as previously described (184). Highbinding flat bottom 96-well plates were coated with 0.2μg of purified CS protein per well in a volume of 100μL and incubated overnight at 4C. Plates were washed with PBS-Tween (0.05%) then blocked with blocking buffer (3% bovine serum albumin) for 1 hour at room temperature. Plasma was diluted (1:50, 1:100, 1:200, 1:400) in blocking buffer and added to the wells and incubated for 1 hour at room temperature. Wells were washed with PBS-Tween (0.05%) and HRP antibody (Bio-Rad) was added at 1:4000 dilution in PBS-Tween. Tetramethylbenzidine (TMB) (Sigma-Aldich) was added to each well and the reaction was stopped with 1N phosphoric acid. Plates are read at 450nm in a microplate spectrophotometer. Subisotyping tittering was completed with a hybridoma subisotyping kit (Calbiochem, La Jolla, CA) with plasma dilutions of 1:50, 1:100. 1:200. 1:400. Statistical analyses were performed using Student t-test. 6.15. Western blotting Spleen and liver tissues were homogenized in lysis buffer (20mM Tris-HCl, pH 7.4, 1mM EDTA, 150mM NaCl) containing 1% Triton X-100 with protease inhibitors. Homogenized tissues were then centrifuged at maximum speed (13,000×G) for 10 min at 4°C, after which the protein concentration of the supernatant determined using BCA method. Western blotting for pErk1/2 and Erk2 was performed as previously described (184). Equivalent concentrations of protein samples were run on polyacrylamide gels and transferred onto nitrocellulose membranes. 220 Blots were then probed with fluorescent antibodies as previously described, which included antibodies against pErk1/2 (Cell Signaling, Inc., Boston, MA) and Erk2 (Santa Cruz Biotechnologies, Santa Cruz, CA). Blots were scanned and bands were quantified using Licor’s Odyssey scanner. For data analysis, the fluorescence of pErk1/2 bands was normalized to Erk2 bands prior to quantification. 6.16. Statistical analysis For every experiment, pilot trials were performed with 3 mice per group (or N=3 for in vitro experiments). This allowed us to determine effect size and sample variance so that Power Analysis could be performed to correctly determine the number of subjects per group required to achieve a statistical Power > 0.8 at the 95% confidence level. Statistically significant differences in toxicities associated with innate immune response (i.e. proinflammatory cytokines, gene induction, etc.) were determined using One Way ANOVA with a Student-Newman-Keuls posthoc test (p value < 0.05). Furthermore, a Two Way ANOVA with a Bonferroni post-hoc test was used to analyze the levels of cytokines at 1 and 6 hpi (or other specified time points) to determine significant differences (p value < 0.05) between groups. Statistically significant differences in ELISpot assays were determined using One Way ANOVA with a Student-Newman-Keuls posthoc test (p value < 0.05). For ELISA analysis, a t-test was used to assess significance between treatments. For multi-parameter flow cytometry, a One Way ANOVA with a Student-NewmanKeuls post-hoc test was used. For in vivo CTL assay, a One Way ANOVA with a StudentNewman-Keuls post-hoc test was used. Furthermore, a Two Way ANOVA with a Bonferroni post-hoc test was used to analyze the effect of pharmacological inhibitors (p value < 0.05). For western blot analysis, a two tailed Student’s t-test was used to compare two groups of virus- 221 infected cells. All graphs are presented as Mean of the average ± SD. GraphPad Prism software was utilized for statistical analysis. 222 Chapter VII Overall Summary and Significance Agents that stimulate the innate immune system, generally referred to as immune adjuvants, or simply adjuvants, have received attention as tools for designing more potent vaccine platforms. Harnessing innate immunity therefore offers considerable promise in designing the next generation of vaccine candidates. The molecular mechanism of action of many adjuvants remained poorly understood. Several studies however have shown that aluminum salts, the only FDA approved adjuvant, functions primarily by activating the innate immune system by promoting recruitment and increasing antigen uptake by APCs, inducing cytokine and chemokine secretions, and enhancing the expression of adhesion molecules involved in migration of leukocytes (129). Our studies in Chapters II and III have shown that, the Eimeria tenella derived protein, rEA; possess all the properties of an ideal immunologic adjuvant. The rEA protein was previously isolated from bovine small intestinal extracts and was shown to be a potent stimulator of innate immune responses in various mouse models in vitro and in vivo, and to also have remarkable anti-viral and anti-cancer activity (131). Administrations of rEA have been shown to be safe and very well-tolerated in human clinical trials (432). We previously described a new Ad-based vaccine vector that expresses the immunomodulatory TLR agonist, rEA (133). We have proven that synergistic manipulation of TLR dependent innate immune responses by rEA improve the ability of Ad-based vaccines to induce beneficial immune responses to pathogen derived antigens, HIV-Gag (133). The molecular mechanism by which rEA functions as an adjuvant is not completely understood, however our data suggested that rEA enhanced release of 223 pro-inflammatory cyto/chemokines from antigen presenting cells as well enhancing their maturational characteristics may facilitate more efficient antigen processing and MHC class I and II antigen presentation so as to improve antigen specific T cell (CD8+) responses. In attempts to investigate the molecular mechanism underlying rEA adjuvant activity, we confirmed that rEA rapidly activates multiple immune cell types, including DCs, macrophages, NK-, NKT-, B-, and T-cells. The rEA adjuvant also elicits the induction of pleiotropic pro-inflammatory cytokines and chemokines responses, responses that completely depend upon the presence of the TLR adaptor protein MyD88. During the course of this work, we were the first to identify that the TRIF adaptor protein acts as a potent negative regulator of TLR (rEA) agonist-triggered immune responses in mice. Our studies further confirmed that the TRIF suppressive activity was not restricted to rEA-mediated responses, but were also apparent when TLR4 (LPS), TLR7/8 (R848) or TLR9 (ODN2006) agonists were used to stimulate DCs as well, thereby unveiling the potential complexities of modulating TLR activity to augment vaccine efficacy. Future studies could further expand upon these findings. For example, as our studies confirmed that DCs are the main subset of innate immune cells that mediate rEA-triggered responses, future studies should determine if ex vivo isolation of DCs and ex vivo exposure of the DCs to antigens and to rEA could improve the potential use of rEA adjuvant in this DC-vaccine setting. Furthermore, rEA has been shown to have adjuvant activity in human cells, which do not express the TLR receptor that has been proposed for profilin molecules that are similar to rEA, such as TLR11/12. Future studies will be required to investigate the innate immune receptor, as well as the innate immune cell type, that rEA triggers to activate human immune cells. The ability to easily scale up Ad5 vector production has resulted in thousands of patients safely receiving recombinant, cGMP compliant, Ad5 based gene transfer vectors. The large 224 number of patients safely treated with the Ad5 platform in vaccine applications further supports its high likelihood for acceptance by regulatory bodies, relative to less well tested platforms, such as alternative Ad serotype derived vaccines, and/or other virus based vaccine platforms. Ad vectors are being explored as potential vaccine candidates for a variety of pathogens. Reports from experimental animal systems have shown that Ad5 vectors induce potent transgene + product-specific antibody and CD8 T cell responses in rodents, dogs, and nonhuman primates (169, 433). For example, Ad vectors encoding a variety of antigens (such as, HIV/Gag, CSP, and CEA) have an enhanced antigen presentation capacity and the ability to induce antigen specific CTL responses (208). Further, E1 deleted Ad5 vectors expressing the HIV-1 gag, pol and nef genes have been utilized in human trial (Merck® sponsored STEP trial). Despite the ability of first generation rAd vectors to induce cellular and humoral immune responses in humans, the results derived from the STEP trial suggest that a more potent vaccine capable of inducing greater levels of antigen specific adaptive immune responses may demonstrate greater efficacy to prevent HIV infection. These facts indicate a need for development of more efficacious Adbased vaccine vectors for this, and many other highly stringent clinical applications. Several lines of investigation suggest that the induction of innate immune responses, (i.e.: TLR-mediated signaling) may be an important reason as to why Ad based vaccines appear to be superior to other vaccine platforms, especially in regard to elicitation of antigen specific cellular immune responses. To describe the adjuvant characteristics of rAd5 vectors, we have reviewed in detail the ability of rAd5 vectors to induce potent innate and adaptive immune responses to the vector as well to the transgene it express. We have also reviewed several attempts to improve rAd5-based vaccine platforms for the development of next generation vaccine candidates. Utilization of advanced generation Ad vectors have been shown to allow for improved efficacy 225 in several vaccine based applications (434). In this dissertation, we have introduced a new strategy for engineering rAd5 vectors, namely by enhancing beneficial innate immune responses subsequent to rAd5 vaccination in an effort to improve the adaptive immune responses to the coadministered antigens. Specifically, we confirmed that targeted manipulation of intra-cellular signaling initiated by the SLAM family of receptors, via Ad mediated co-expression of the SLAM adaptor protein EAT-2 adaptor facilitated improved induction of several arms of the innate immune system, and that these inductions positively correlated with an improved ability of Ad vaccine formulations expressing EAT-2 to induce stronger cellular immune responses to Ad vaccine expressed antigens. Ad5 vectors expressing EAT-2 facilitate bystander activation of + + NK, NKT, B, CD4 , and CD8 T cells early after their administration into animals. EAT-2 overexpression also augments the expression of surface markers associated with the enhanced function of antigen presenting cells, such as CD40, CD80, CCR7, and CD86. This multi-tiered activation of the innate immune system by vaccine mediated EAT-2 expression also enhanced the induction of Ad vaccine expressed antigens, such as the HIV-Gag antigen, as multiply confirmed by Tetramer-based flow cytometry, ELISPOT, and in vivo CTL assays. Since both mice and humans express highly conserved EAT-2 adaptors, our results suggest that human vaccination strategies that specifically facilitate SLAM signaling may improve vaccine potency when targeting HIV-1 antigens specifically, as well as numerous other vaccine targets in general. In an innovative additional use of this novel vaccine platform, we utilized rAd5-EAT2 vaccine vectors for the development of rAd5-based vaccine that specifically target peptide sequences derived from the malaria causing parasite, P. falciparum. Malaria is an infectious disease that continues to devastate population world-wide, causing nearly 1 million deaths annually, and morbidity that overwhelms the medical capabilities of developing countries. Some 226 of the most successful malaria vaccines studied to date have attempted to induce adaptive immune responses to the P. falciparum CS protein, a protein that is found on the surface of P. falciparum sporozoites and is also expressed by the parasite in hepatocytes during liver infection. The induction of potent cellular immune responses to CS protein by a prophylactic malaria vaccine could potentially eradicate both sporozoites and infected hepatocytes, preventing the infection before clinical symptoms occur. In our studies to improve the immunogenicity of CS expressing Ad vaccines, we confirmed the presence of a potent immunosuppressive activity for the P. falciparum CS protein, when excessive TLR activation by advanced generation Ad vaccines (Ad vaccine formulations expressing rEA) were utilized to enhance CS specific adaptive immune responses. Future studies will need to be performed to elucidate the molecular mechanisms as to how the CS protein suppresses immune responses to CS in the context of excessive TLR activation. Our data suggest that the use of CS protein along with other immunostimulatory compounds, such as TLR agonists, in certain malaria vaccine formulations will have to be carefully considered. In contrast, triggering SLAM family of receptors signaling by Ad vaccine mediated expression of EAT-2 circumvented CS protein’s suppressive activity, and generated a potent increase in the ability of Ad vaccines expressing CS to induce CS protein-specific T cell immune responses. Our findings suggest that augmentation of SLAM initiated signaling and downstream inductions of specific cyto/chemokines may be the mechanism underlying EAT-2's ability to improve induction of antigen specific CTL immune responses. The biochemical mechanism and intracellular signaling pathway behind EAT-2's ability to function as a T cell stimulator is not fully elucidated, but is a question that has been partially unveiled by our studies. 227 In Chapter V, we describe initial investigations as to the molecular mechanism underlying how EAT-2 overexpression can facilitate improved induction of antigen specific adaptive immune responses by Ad based vaccines. We found that EAT-2 over-expression specifically prevents Ad vaccine induced CRACC receptor up-regulation on APCs, a phenomenon that requires a functional ERK MAPK signaling pathway. We have also demonstrated that, utilization of a mutated SH-2 domain form of EAT-2, EAT-2(R31Q), adaptor failed to prevent Ad vaccine induced CRACC receptor up-regulation, suggesting a role for the interaction between EAT-2 SH-2 domains and the phosphorylated ITSMs of SLAM family members. The significance of EAT-2 to prevent Ad mediated up regulation of the CRACC receptor on APCs needs to be further elucidated. Recently, it has been shown that the CRACC receptor functions as an inhibitory molecule in T cells (144). This inhibitory activity is due to homophilic CRAC-CRACC interactions between CRACC receptors on APCs and CRACC receptors on T cells during their interactions, and specifically at the immunological synapse. This homophilic interaction results in phosphorylation of the T cell CRACC receptor ITSMs, and results in the recruitment of inhibitory phosphatases (SHP-1, SHP-2, and Csk) and SHIP-1 that contain SH-2 domains. As a result of this inhibition, T cell proliferation, as well as IL-2 and IFNγ secretion by the T cells in response to antigen presentations by APCs were abrogated. Our studies suggest that expression of EAT-2 may have improved the adaptive immune responses by preventing upregulation of CRACC by Ad vaccines transduced APCs, thereby preventing T cell suppression by excessive CRACC stimulation. Future studies using isolated T cells and DCs, coupled with specific inhibition of CRACC-CRACC interactions (i.e.: via use of CRACC-blocking antibodies) could further validate these hypotheses. We also demonstrated that EAT-2 over-expression regulates CRACC receptor expression at the transcriptional level, 228 suggesting the role for EAT2 responsive transcription factors. Future studies need to be performed to elucidate this mechanism as well, such as CHIP array based analysis. We also demonstrated that Ad mediated EAT-2 expression regulated the production of several cytokines and chemokines, responses that were partially dependent upon the presence of a functional ERK-MAPK signaling pathway. Future studies need to be performed to investigate if one (or more) of these cyto/chemokines may be acting in an autocrine fashion to downregulate CRACC expression in APCs, potentially via use of neutralizing antibodies or recombinant proteins assays. Proving such a role would not only shed light on how EAT-2 signaling facilitates CRACC down-regulation, but would also foster future studies to delineate the mechanisms underlying regulation of CRACC expression in general. We leave our readers with the view that the interactions between the innate and adaptive immune system are multifaceted and complex. It is also clear that despite this level of complexity, harnessing specific arms of the innate immune system, such as the SLAM or TLR signaling dependent pathways, respectively, can have an important impact on the subsequent induction of downstream, antigen specific adaptive immune responses. We predict a continued expansion in the search for safe and efficacious vaccine adjuvants; as well inclusion of pharmacological and/or other supportive interventions to further enhance both the safety and efficacy of human vaccines. 229 BIBLIOGRAPHY 230 BIBLIOGRAPHY 1. Hoffmann JA, Kafatos FC, Janeway CA, & Ezekowitz RA (1999) Phylogenetic perspectives in innate immunity. (Translated from eng) Science 284(5418):13131318 (in eng). 2. Iwasaki A & Medzhitov R (2010) Regulation of adaptive immunity by the innate immune system. (Translated from eng) Science 327(5963):291-295 (in eng). 3. Girardin SE, Sansonetti PJ, & Philpott DJ (2002) Intracellular vs extracellular recognition of pathogens--common concepts in mammals and flies. (Translated from eng) Trends Microbiol 10(4):193-199 (in eng). 4. Kawai T & Akira S (2010) The role of pattern-recognition receptors in innate immunity: update on Toll-like receptors. (Translated from eng) Nat Immunol 11(5):373-384 (in eng). 5. Beutler BA (2009) TLRs and innate immunity. (Translated from eng) Blood 113(7):1399-1407 (in eng). 6. O'Neill LA, Bryant CE, & Doyle SL (2009) Therapeutic targeting of Toll-like receptors for infectious and inflammatory diseases and cancer. (Translated from eng) Pharmacol Rev 61(2):177-197 (in eng). 7. Medzhitov R (2007) Recognition of microorganisms and activation of the immune response. (Translated from eng) Nature 449(7164):819-826 (in eng). 8. Lee MS & Kim YJ (2007) Signaling pathways downstream of pattern-recognition receptors and their cross talk. (Translated from eng) Annu Rev Biochem 76:447-480 (in eng). 9. Takeuchi O & Akira S (2010) Pattern recognition receptors and inflammation. (Translated from eng) Cell 140(6):805-820 (in eng). 10. Kabelitz D & Medzhitov R (2007) Innate immunity--cross-talk with adaptive immunity through pattern recognition receptors and cytokines. (Translated from eng) Curr Opin Immunol 19(1):1-3 (in eng). 11. Imler JL & Zheng L (2004) Biology of Toll receptors: lessons from insects and mammals. (Translated from eng) J Leukoc Biol 75(1):18-26 (in eng). 231 12. Kawai T & Akira S (2007) Antiviral signaling through pattern recognition receptors. (Translated from eng) J Biochem 141(2):137-145 (in eng). 13. Takeda K & Akira S (2005) Toll-like receptors in innate immunity. (Translated from eng) Int Immunol 17(1):1-14 (in eng). 14. O'Neill LA & Bowie AG (2007) The family of five: TIR-domain-containing adaptors in Toll-like receptor signalling. (Translated from eng) Nat Rev Immunol 7(5):353-364 (in eng). 15. O'Neill LA & Bowie AG (2010) Sensing and signaling in antiviral innate immunity. (Translated from eng) Curr Biol 20(7):R328-333 (in eng). 16. Bonilla FA & Oettgen HC (2010) Adaptive immunity. (Translated from eng) J Allergy Clin Immunol 125(2 Suppl 2):S33-40 (in eng). 17. Hedrick SM (2008) Thymus lineage commitment: a single switch. (Translated from eng) Immunity 28(3):297-299 (in eng). 18. LeBien TW & Tedder TF (2008) B lymphocytes: how they develop and function. (Translated from eng) Blood 112(5):1570-1580 (in eng). 19. Cyster JG, Hartley SB, & Goodnow CC (1994) Competition for follicular niches excludes self-reactive cells from the recirculating B-cell repertoire. (Translated from eng) Nature 371(6496):389-395 (in eng). 20. Ma D, Wei Y, & Liu F (2011) Regulatory mechanisms of thymus and T cell development. (Translated from Eng) Dev Comp Immunol (in Eng). 21. Gallegos AM & Bevan MJ (2006) Central tolerance: good but imperfect. (Translated from eng) Immunol Rev 209:290-296 (in eng). 22. Macian F, Im SH, Garcia-Cozar FJ, & Rao A (2004) T-cell anergy. (Translated from eng) Curr Opin Immunol 16(2):209-216 (in eng). 23. Sakaguchi S (2011) Regulatory T cells: history and perspective. (Translated from eng) Methods Mol Biol 707:3-17 (in eng). 24. Gonzalez SF, et al. (2011) Trafficking of B cell antigen in lymph nodes. (Translated from eng) Annu Rev Immunol 29:215-233 (in eng). 25. Smith-Garvin JE, Koretzky GA, & Jordan MS (2009) T cell activation. (Translated from eng) Annu Rev Immunol 27:591-619 (in eng). 26. Michalek RD & Rathmell JC (2010) The metabolic life and times of a T-cell. (Translated from eng) Immunol Rev 236:190-202 (in eng). 232 27. Zhang N & Bevan MJ (2011) CD8(+) T cells: foot soldiers of the immune system. (Translated from eng) Immunity 35(2):161-168 (in eng). 28. Powell JD & Delgoffe GM (2010) The mammalian target of rapamycin: linking T cell differentiation, function, and metabolism. (Translated from eng) Immunity 33(3):301-311 (in eng). 29. Le Bon A, et al. (2006) Direct stimulation of T cells by type I IFN enhances the CD8+ T cell response during cross-priming. (Translated from eng) J Immunol 176(8):4682-4689 (in eng). 30. Pearce EL & Shen H (2007) Generation of CD8 T cell memory is regulated by IL-12. (Translated from eng) J Immunol 179(4):2074-2081 (in eng). 31. Williams MA, Tyznik AJ, & Bevan MJ (2006) Interleukin-2 signals during priming are required for secondary expansion of CD8+ memory T cells. (Translated from eng) Nature 441(7095):890-893 (in eng). 32. Pipkin ME, et al. (2010) Interleukin-2 and inflammation induce distinct transcriptional programs that promote the differentiation of effector cytolytic T cells. (Translated from eng) Immunity 32(1):79-90 (in eng). 33. Quigley M, Martinez J, Huang X, & Yang Y (2009) A critical role for direct TLR2MyD88 signaling in CD8 T-cell clonal expansion and memory formation following vaccinia viral infection. (Translated from eng) Blood 113(10):2256-2264 (in eng). 34. Groom JR & Luster AD (2011) CXCR3 ligands: redundant, collaborative and antagonistic functions. (Translated from eng) Immunol Cell Biol 89(2):207-215 (in eng). 35. Trandem K, Zhao J, Fleming E, & Perlman S (2011) Highly activated cytotoxic CD8 T cells express protective IL-10 at the peak of coronavirus-induced encephalitis. (Translated from eng) J Immunol 186(6):3642-3652 (in eng). 36. Kaech SM, et al. (2003) Selective expression of the interleukin 7 receptor identifies effector CD8 T cells that give rise to long-lived memory cells. (Translated from eng) Nat Immunol 4(12):1191-1198 (in eng). 37. Parish IA & Kaech SM (2009) Diversity in CD8(+) T cell differentiation. (Translated from eng) Curr Opin Immunol 21(3):291-297 (in eng). 38. Wherry EJ, et al. (2003) Lineage relationship and protective immunity of memory CD8 T cell subsets. (Translated from eng) Nat Immunol 4(3):225-234 (in eng). 233 39. Gattinoni L, et al. (2009) Wnt signaling arrests effector T cell differentiation and generates CD8+ memory stem cells. (Translated from eng) Nat Med 15(7):808-813 (in eng). 40. Araki K, et al. (2009) mTOR regulates memory CD8 T-cell differentiation. (Translated from eng) Nature 460(7251):108-112 (in eng). 41. Surh CD & Sprent J (2008) Homeostasis of naive and memory T cells. (Translated from eng) Immunity 29(6):848-862 (in eng). 42. Zhu J, Yamane H, & Paul WE (2010) Differentiation of effector CD4 T cell populations (*). (Translated from eng) Annu Rev Immunol 28:445-489 (in eng). 43. Mosmann TR, Cherwinski H, Bond MW, Giedlin MA, & Coffman RL (1986) Two types of murine helper T cell clone. I. Definition according to profiles of lymphokine activities and secreted proteins. (Translated from eng) J Immunol 136(7):2348-2357 (in eng). 44. Sallusto F & Lanzavecchia A (2009) Heterogeneity of CD4+ memory T cells: functional modules for tailored immunity. (Translated from eng) Eur J Immunol 39(8):2076-2082 (in eng). 45. Cua DJ, et al. (2003) Interleukin-23 rather than interleukin-12 is the critical cytokine for autoimmune inflammation of the brain. (Translated from eng) Nature 421(6924):744-748 (in eng). 46. Milner JD, et al. (2008) Impaired T(H)17 cell differentiation in subjects with autosomal dominant hyper-IgE syndrome. (Translated from eng) Nature 452(7188):773-776 (in eng). 47. Veldhoen M, et al. (2008) Transforming growth factor-beta 'reprograms' the differentiation of T helper 2 cells and promotes an interleukin 9-producing subset. (Translated from eng) Nat Immunol 9(12):1341-1346 (in eng). 48. Zheng Y, et al. (2007) Interleukin-22, a T(H)17 cytokine, mediates IL-23-induced dermal inflammation and acanthosis. (Translated from eng) Nature 445(7128):648651 (in eng). 49. King C, Tangye SG, & Mackay CR (2008) T follicular helper (TFH) cells in normal and dysregulated immune responses. (Translated from eng) Annu Rev Immunol 26:741766 (in eng). 50. O'Garra A, Vieira PL, Vieira P, & Goldfeld AE (2004) IL-10-producing and naturally occurring CD4+ Tregs: limiting collateral damage. (Translated from eng) J Clin Invest 114(10):1372-1378 (in eng). 234 51. Belkaid Y & Tarbell K (2009) Regulatory T cells in the control of hostmicroorganism interactions (*). (Translated from eng) Annu Rev Immunol 27:551589 (in eng). 52. von Boehmer H (2005) Mechanisms of suppression by suppressor T cells. (Translated from eng) Nat Immunol 6(4):338-344 (in eng). 53. Belkaid Y, et al. (2001) The role of interleukin (IL)-10 in the persistence of Leishmania major in the skin after healing and the therapeutic potential of anti-IL10 receptor antibody for sterile cure. (Translated from eng) J Exp Med 194(10):1497-1506 (in eng). 54. McKinley L, et al. (2006) Regulatory T cells dampen pulmonary inflammation and lung injury in an animal model of pneumocystis pneumonia. (Translated from eng) J Immunol 177(9):6215-6226 (in eng). 55. Hobeika AC, et al. (2011) Depletion of human regulatory T cells. (Translated from eng) Methods Mol Biol 707:219-231 (in eng). 56. Harwood NE & Batista FD (2008) New insights into the early molecular events underlying B cell activation. (Translated from eng) Immunity 28(5):609-619 (in eng). 57. Depoil D, et al. (2008) CD19 is essential for B cell activation by promoting B cell receptor-antigen microcluster formation in response to membrane-bound ligand. (Translated from eng) Nat Immunol 9(1):63-72 (in eng). 58. Fearon DT & Carroll MC (2000) Regulation of B lymphocyte responses to foreign and self-antigens by the CD19/CD21 complex. (Translated from eng) Annu Rev Immunol 18:393-422 (in eng). 59. Nutt SL, Heavey B, Rolink AG, & Busslinger M (1999) Commitment to the Blymphoid lineage depends on the transcription factor Pax5. (Translated from eng) Nature 401(6753):556-562 (in eng). 60. Cobaleda C, Schebesta A, Delogu A, & Busslinger M (2007) Pax5: the guardian of B cell identity and function. (Translated from eng) Nat Immunol 8(5):463-470 (in eng). 61. Mullighan CG, et al. (2007) Genome-wide analysis of genetic alterations in acute lymphoblastic leukaemia. (Translated from eng) Nature 446(7137):758-764 (in eng). 62. Allen CD, Okada T, & Cyster JG (2007) Germinal-center organization and cellular dynamics. (Translated from eng) Immunity 27(2):190-202 (in eng). 235 63. Radbruch A, et al. (2006) Competence and competition: the challenge of becoming a long-lived plasma cell. (Translated from eng) Nat Rev Immunol 6(10):741-750 (in eng). 64. Montecino-Rodriguez E, Leathers H, & Dorshkind K (2006) Identification of a B-1 B cell-specified progenitor. (Translated from eng) Nat Immunol 7(3):293-301 (in eng). 65. Haas KM, Poe JC, Steeber DA, & Tedder TF (2005) B-1a and B-1b cells exhibit distinct developmental requirements and have unique functional roles in innate and adaptive immunity to S. pneumoniae. (Translated from eng) Immunity 23(1):7-18 (in eng). 66. Steiniger B, Timphus EM, & Barth PJ (2006) The splenic marginal zone in humans and rodents: an enigmatic compartment and its inhabitants. (Translated from eng) Histochem Cell Biol 126(6):641-648 (in eng). 67. Matsushita T & Tedder TF (2011) Identifying regulatory B cells (B10 cells) that produce IL-10 in mice. (Translated from eng) Methods Mol Biol 677:99-111 (in eng). 68. Iwasaki A & Medzhitov R (2004) Toll-like receptor control of the adaptive immune responses. (Translated from eng) Nat Immunol 5(10):987-995 (in eng). 69. Leon B & Ardavin C (2008) Monocyte-derived dendritic cells in innate and adaptive immunity. (Translated from eng) Immunol Cell Biol 86(4):320-324 (in eng). 70. Reschner A, Hubert P, Delvenne P, Boniver J, & Jacobs N (2008) Innate lymphocyte and dendritic cell cross-talk: a key factor in the regulation of the immune response. (Translated from eng) Clin Exp Immunol 152(2):219-226 (in eng). Ferlazzo G, et al. (2004) Distinct roles of IL-12 and IL-15 in human natural killer cell activation by dendritic cells from secondary lymphoid organs. (Translated from eng) Proc Natl Acad Sci U S A 101(47):16606-16611 (in eng). 71. 72. Lopez-Bravo M & Ardavin C (2008) In vivo induction of immune responses to pathogens by conventional dendritic cells. (Translated from eng) Immunity 29(3):343-351 (in eng). 73. Mount AM, et al. (2008) Multiple dendritic cell populations activate CD4+ T cells after viral stimulation. (Translated from eng) PLoS One 3(2):e1691 (in eng). 74. Allan RS, et al. (2006) Migratory dendritic cells transfer antigen to a lymph noderesident dendritic cell population for efficient CTL priming. (Translated from eng) Immunity 25(1):153-162 (in eng). 75. Fallarino F, et al. (2004) Murine plasmacytoid dendritic cells initiate the immunosuppressive pathway of tryptophan catabolism in response to CD200 receptor engagement. (Translated from eng) J Immunol 173(6):3748-3754 (in eng). 236 76. Piccioli D, Sbrana S, Melandri E, & Valiante NM (2002) Contact-dependent stimulation and inhibition of dendritic cells by natural killer cells. (Translated from eng) J Exp Med 195(3):335-341 (in eng). 77. Piccioli D, Sbrana S, Melandri E, & Valiante NM (2002) Contact-dependent stimulation and inhibition of dendritic cells by natural killer cells. (Translated from eng) The Journal of experimental medicine 195(3):335-341 (in eng). 78. Martin-Fontecha A, et al. (2004) Induced recruitment of NK cells to lymph nodes provides IFN-gamma for T(H)1 priming. (Translated from eng) Nature immunology 5(12):1260-1265 (in eng). 79. Kelly JM, et al. (2002) Induction of tumor-specific T cell memory by NK cellmediated tumor rejection. (Translated from eng) Nat Immunol 3(1):83-90 (in eng). 80. Krebs P, et al. (2009) NK-cell-mediated killing of target cells triggers robust antigenspecific T-cell-mediated and humoral responses. (Translated from eng) Blood 113(26):6593-6602 (in eng). 81. Gao N, Jennings P, & Yuan D (2008) Requirements for the natural killer cellmediated induction of IgG1 and IgG2a expression in B lymphocytes. (Translated from eng) Int Immunol 20(5):645-657 (in eng). 82. Winkler-Pickett R, et al. (2008) In vivo regulation of experimental autoimmune encephalomyelitis by NK cells: alteration of primary adaptive responses. (Translated from eng) J Immunol 180(7):4495-4506 (in eng). 83. Su HC, et al. (2001) NK cell functions restrain T cell responses during viral infections. (Translated from eng) Eur J Immunol 31(10):3048-3055 (in eng). 84. Barber MA, Zhang T, Gagne BA, & Sentman CL (2007) NK cells negatively regulate antigen presentation and tumor-specific CTLs in a syngeneic lymphoma model. (Translated from eng) J Immunol 178(10):6140-6147 (in eng). 85. Fujii S, Shimizu K, Smith C, Bonifaz L, & Steinman RM (2003) Activation of natural killer T cells by alpha-galactosylceramide rapidly induces the full maturation of dendritic cells in vivo and thereby acts as an adjuvant for combined CD4 and CD8 T cell immunity to a coadministered protein. (Translated from eng) The Journal of experimental medicine 198(2):267-279 (in eng). 86. Hermans IF, et al. (2003) NKT cells enhance CD4+ and CD8+ T cell responses to soluble antigen in vivo through direct interaction with dendritic cells. (Translated from eng) J Immunol 171(10):5140-5147 (in eng). 87. Cerundolo V, Silk JD, Masri SH, & Salio M (2009) Harnessing invariant NKT cells in vaccination strategies. (Translated from eng) Nature reviews 9(1):28-38 (in eng). 237 88. Hermans IF, et al. (2007) Dendritic cell function can be modulated through cooperative actions of TLR ligands and invariant NKT cells. (Translated from eng) J Immunol 178(5):2721-2729 (in eng). 89. Dondji B, et al. (2008) Intradermal NKT cell activation during DNA priming in heterologous prime-boost vaccination enhances T cell responses and protection against Leishmania. (Translated from eng) European journal of immunology 38(3):706-719 (in eng). 90. Mantovani A, Cassatella MA, Costantini C, & Jaillon S (2011) Neutrophils in the activation and regulation of innate and adaptive immunity. (Translated from eng) Nat Rev Immunol 11(8):519-531 (in eng). 91. Bennouna S & Denkers EY (2005) Microbial antigen triggers rapid mobilization of TNF-alpha to the surface of mouse neutrophils transforming them into inducers of high-level dendritic cell TNF-alpha production. (Translated from eng) J Immunol 174(8):4845-4851 (in eng). 92. Costantini C, et al. (2011) Human neutrophils interact with both 6-sulfo LacNAc+ DC and NK cells to amplify NK-derived IFN{gamma}: role of CD18, ICAM-1, and ICAM-3. (Translated from eng) Blood 117(5):1677-1686 (in eng). 93. Nathan C (2006) Neutrophils and immunity: challenges and opportunities. (Translated from eng) Nat Rev Immunol 6(3):173-182 (in eng). 94. Scapini P, Bazzoni F, & Cassatella MA (2008) Regulation of B-cell-activating factor (BAFF)/B lymphocyte stimulator (BLyS) expression in human neutrophils. (Translated from eng) Immunol Lett 116(1):1-6 (in eng). 95. Pelletier M, et al. (2010) Evidence for a cross-talk between human neutrophils and Th17 cells. (Translated from eng) Blood 115(2):335-343 (in eng). 96. Abi Abdallah DS, Egan CE, Butcher BA, & Denkers EY (2011) Mouse neutrophils are professional antigen-presenting cells programmed to instruct Th1 and Th17 T-cell differentiation. (Translated from eng) Int Immunol 23(5):317-326 (in eng). 97. Morgan BP, Marchbank KJ, Longhi MP, Harris CL, & Gallimore AM (2005) Complement: central to innate immunity and bridging to adaptive responses. (Translated from eng) Immunol Lett 97(2):171-179 (in eng). 98. Carroll MC (2004) The complement system in regulation of adaptive immunity. (Translated from eng) Nat Immunol 5(10):981-986 (in eng). 99. Carroll MC (2004) The complement system in B cell regulation. (Translated from eng) Mol Immunol 41(2-3):141-146 (in eng). 238 100. Molina H, et al. (1996) Markedly impaired humoral immune response in mice deficient in complement receptors 1 and 2. (Translated from eng) Proc Natl Acad Sci U S A 93(8):3357-3361 (in eng). 101. Seregin SS, et al. (2009) CR1/2 is an important suppressor of Adenovirus-induced innate immune responses and is required for induction of neutralizing antibodies. (Translated from eng) Gene Ther 16(10):1245-1259 (in eng). 102. Seregin SS, et al. (2010) Adenovirus capsid-display of the retro-oriented human complement inhibitor DAF reduces Ad vector-triggered immune responses in vitro and in vivo. (Translated from eng) Blood 116(10):1669-1677 (in eng). 103. Seregin SS, et al. (2011) Use of DAF-Displaying Adenovirus Vectors Reduces Induction of Transgene- and Vector-Specific Adaptive Immune Responses in Mice. (Translated from Eng) Hum Gene Ther (in Eng). 104. Soruri A, et al. (2003) Anaphylatoxin C5a induces monocyte recruitment and differentiation into dendritic cells by TNF-alpha and prostaglandin E2-dependent mechanisms. (Translated from eng) J Immunol 171(5):2631-2636 (in eng). 105. Kawamoto S, et al. (2004) The anaphylatoxin C3a downregulates the Th2 response to epicutaneously introduced antigen. (Translated from eng) J Clin Invest 114(3):399-407 (in eng). 106. Takahara M, et al. (2003) iC3b arrests monocytic cell differentiation into CD1cexpressing dendritic cell precursors: a mechanism for transiently decreased dendritic cells in vivo after human skin injury by ultraviolet B. (Translated from eng) J Invest Dermatol 120(5):802-809 (in eng). 107. Kumar H, Kawai T, & Akira S (2009) Toll-like receptors and innate immunity. (Translated from eng) Biochem Biophys Res Commun 388(4):621-625 (in eng). 108. Pulendran B & Ahmed R (2006) Translating innate immunity into immunological memory: implications for vaccine development. (Translated from eng) Cell 124(4):849-863 (in eng). 109. Kolumam GA, Thomas S, Thompson LJ, Sprent J, & Murali-Krishna K (2005) Type I interferons act directly on CD8 T cells to allow clonal expansion and memory formation in response to viral infection. (Translated from eng) J Exp Med 202(5):637-650 (in eng). 110. Dillon S, et al. (2004) A Toll-like receptor 2 ligand stimulates Th2 responses in vivo, via induction of extracellular signal-regulated kinase mitogen-activated protein kinase and c-Fos in dendritic cells. (Translated from eng) J Immunol 172(8):47334743 (in eng). 239 111. Brown GD & Gordon S (2001) Immune recognition. A new receptor for beta-glucans. (Translated from eng) Nature 413(6851):36-37 (in eng). 112. LeibundGut-Landmann S, et al. (2007) Syk- and CARD9-dependent coupling of innate immunity to the induction of T helper cells that produce interleukin 17. (Translated from eng) Nat Immunol 8(6):630-638 (in eng). 113. Williams A, Flavell RA, & Eisenbarth SC (2010) The role of NOD-like Receptors in shaping adaptive immunity. (Translated from eng) Curr Opin Immunol 22(1):34-40 (in eng). 114. Fritz JH, et al. (2007) Nod1-mediated innate immune recognition of peptidoglycan contributes to the onset of adaptive immunity. (Translated from eng) Immunity 26(4):445-459 (in eng). 115. Kobayashi KS, et al. (2005) Nod2-dependent regulation of innate and adaptive immunity in the intestinal tract. (Translated from eng) Science 307(5710):731-734 (in eng). 116. Schroder K & Tschopp J (2010) The inflammasomes. (Translated from eng) Cell 140(6):821-832 (in eng). 117. Ghiringhelli F, et al. (2009) Activation of the NLRP3 inflammasome in dendritic cells induces IL-1beta-dependent adaptive immunity against tumors. (Translated from eng) Nat Med 15(10):1170-1178 (in eng). 118. Chiu YH, Macmillan JB, & Chen ZJ (2009) RNA polymerase III detects cytosolic DNA and induces type I interferons through the RIG-I pathway. (Translated from eng) Cell 138(3):576-591 (in eng). 119. Ishii KJ, et al. (2008) TANK-binding kinase-1 delineates innate and adaptive immune responses to DNA vaccines. (Translated from eng) Nature 451(7179):725-729 (in eng). 120. Coffman RL, Sher A, & Seder RA (2010) Vaccine adjuvants: putting innate immunity to work. (Translated from eng) Immunity 33(4):492-503 (in eng). 121. Nemazee D, Gavin A, Hoebe K, & Beutler B (2006) Immunology: Toll-like receptors and antibody responses. (Translated from eng) Nature 441(7091):E4; discussion E4 (in eng). 122. Hoebe K, Janssen E, & Beutler B (2004) The interface between innate and adaptive immunity. (Translated from eng) Nat Immunol 5(10):971-974 (in eng). 240 123. Stahl-Hennig C, et al. (2009) Synthetic double-stranded RNAs are adjuvants for the induction of T helper 1 and humoral immune responses to human papillomavirus in rhesus macaques. (Translated from eng) PLoS Pathog 5(4):e1000373 (in eng). 124. Casella CR & Mitchell TC (2008) Putting endotoxin to work for us: monophosphoryl lipid A as a safe and effective vaccine adjuvant. (Translated from eng) Cell Mol Life Sci 65(20):3231-3240 (in eng). 125. Sanders CJ, Moore DA, 3rd, Williams IR, & Gewirtz AT (2008) Both radioresistant and hemopoietic cells promote innate and adaptive immune responses to flagellin. (Translated from eng) J Immunol 180(11):7184-7192 (in eng). 126. Hemmi H, et al. (2002) Small anti-viral compounds activate immune cells via the TLR7 MyD88-dependent signaling pathway. (Translated from eng) Nat Immunol 3(2):196-200 (in eng). 127. Campbell JD, et al. (2009) CpG-containing immunostimulatory DNA sequences elicit TNF-alpha-dependent toxicity in rodents but not in humans. (Translated from eng) J Clin Invest 119(9):2564-2576 (in eng). 128. Mosca F, et al. (2008) Molecular and cellular signatures of human vaccine adjuvants. (Translated from eng) Proc Natl Acad Sci U S A 105(30):10501-10506 (in eng). 129. Hem SL & Hogenesch H (2007) Relationship between physical and chemical properties of aluminum-containing adjuvants and immunopotentiation. (Translated from eng) Expert Rev Vaccines 6(5):685-698 (in eng). 130. Seubert A, Monaci E, Pizza M, O'Hagan DT, & Wack A (2008) The adjuvants aluminum hydroxide and MF59 induce monocyte and granulocyte chemoattractants and enhance monocyte differentiation toward dendritic cells. (Translated from eng) J Immunol 180(8):5402-5412 (in eng). 131. Rosenberg B, et al. (2005) Protein from intestinal Eimeria protozoan stimulates IL12 release from dendritic cells, exhibits antitumor properties in vivo and is correlated with low intestinal tumorigenicity. (Translated from eng) Int J Cancer 114(5):756-765 (in eng). 132. Seregin SS, et al. (2011) TRIF is a critical negative regulator of TLR agonist mediated activation of dendritic cells in vivo. (Translated from eng) PLoS One 6(7):e22064 (in eng). 133. Appledorn DM, et al. (2010) A new adenovirus based vaccine vector expressing an Eimeria tenella derived TLR agonist improves cellular immune responses to an antigenic target. (Translated from eng) PLoS One 5(3):e9579 (in eng). 241 134. Hedhli D, Dimier-Poisson I, Judge JW, Rosenberg B, & Mevelec MN (2009) Protective immunity against Toxoplasma challenge in mice by coadministration of T. gondii antigens and Eimeria profilin-like protein as an adjuvant. (Translated from eng) Vaccine 27(16):2274-2281 (in eng). 135. Gowen BB, et al. (2006) Recombinant Eimeria protozoan protein elicits resistance to acute phlebovirus infection in mice but not hamsters. (Translated from eng) Antimicrob Agents Chemother 50(6):2023-2029 (in eng). 136. Juckett DA, Aylsworth CF, & Quensen JM (2008) Intestinal protozoa are hypothesized to stimulate immunosurveillance against colon cancer. (Translated from eng) Med Hypotheses 71(1):104-110 (in eng). 137. Rader JS, et al. (2008) Phase I study and preliminary pharmacology of the novel innate immune modulator rBBX-01 in gynecologic cancers. (Translated from eng) Clin Cancer Res 14(10):3089-3097 (in eng). 138. Yarovinsky F, et al. (2005) TLR11 activation of dendritic cells by a protozoan profilin-like protein. (Translated from eng) Science 308(5728):1626-1629 (in eng). 139. Appledorn DM, Aldhamen YA, Godbehere S, Seregin SS, & Amalfitano A (Sublingual administration of an Adenovirus based vaccine confirms TLR agonist activity in the oral cavity and elicits improved mucosal and systemic cell mediated responses against HIV antigens despite pre-existing Ad5 immunity. (Translated from Eng) Clin Vaccine Immunol (in Eng). 140. Veillette A (2010) SLAM-family receptors: immune regulators with or without SAPfamily adaptors. (Translated from eng) Cold Spring Harb Perspect Biol 2(3):a002469 (in eng). 141. Ma CS, Nichols KE, & Tangye SG (2007) Regulation of cellular and humoral immune responses by the SLAM and SAP families of molecules. (Translated from eng) Annu Rev Immunol 25:337-379 (in eng). 142. Latchman Y, McKay PF, & Reiser H (1998) Identification of the 2B4 molecule as a counter-receptor for CD48. (Translated from eng) J Immunol 161(11):5809-5812 (in eng). 143. Wille-Reece U, et al. (2006) Toll-like receptor agonists influence the magnitude and quality of memory T cell responses after prime-boost immunization in nonhuman primates. (Translated from eng) J Exp Med 203(5):1249-1258 (in eng). 144. Cruz-Munoz ME, Dong Z, Shi X, Zhang S, & Veillette A (2009) Influence of CRACC, a SLAM family receptor coupled to the adaptor EAT-2, on natural killer cell function. (Translated from eng) Nat Immunol 10(3):297-305 (in eng). 242 145. Morra M, et al. (2001) X-linked lymphoproliferative disease: a progressive immunodeficiency. (Translated from eng) Annu Rev Immunol 19:657-682 (in eng). 146. Wandstrat AE, et al. (2004) Association of extensive polymorphisms in the SLAM/CD2 gene cluster with murine lupus. (Translated from eng) Immunity 21(6):769-780 (in eng). 147. Suzuki A, et al. (2008) Functional SNPs in CD244 increase the risk of rheumatoid arthritis in a Japanese population. (Translated from eng) Nat Genet 40(10):12241229 (in eng). 148. Cunninghame Graham DS, et al. (2008) Association of LY9 in UK and Canadian SLE families. (Translated from eng) Genes Immun 9(2):93-102 (in eng). 149. Veillette A (2006) Immune regulation by SLAM family receptors and SAP-related adaptors. (Translated from eng) Nat Rev Immunol 6(1):56-66 (in eng). 150. Tassi I & Colonna M (2005) The cytotoxicity receptor CRACC (CS-1) recruits EAT-2 and activates the PI3K and phospholipase Cgamma signaling pathways in human NK cells. (Translated from eng) J Immunol 175(12):7996-8002 (in eng). 151. Cruz-Munoz ME, Dong Z, Shi X, Zhang S, & Veillette A (2009) Influence of CRACC, a SLAM family receptor coupled to the adaptor EAT-2, on natural killer cell function. (Translated from eng) Nat Immunol 10(3):297-305 (in eng). 152. Sayos J, et al. (1998) The X-linked lymphoproliferative-disease gene product SAP regulates signals induced through the co-receptor SLAM. (Translated from eng) Nature 395(6701):462-469 (in eng). 153. Schwartzberg PL, Mueller KL, Qi H, & Cannons JL (2009) SLAM receptors and SAP influence lymphocyte interactions, development and function. (Translated from eng) Nat Rev Immunol 9(1):39-46 (in eng). 154. Veillette A, et al. (2008) SAP expression in T cells, not in B cells, is required for humoral immunity. (Translated from eng) Proc Natl Acad Sci U S A 105(4):12731278 (in eng). 155. Pasquier B, et al. (2005) Defective NKT cell development in mice and humans lacking the adapter SAP, the X-linked lymphoproliferative syndrome gene product. (Translated from eng) J Exp Med 201(5):695-701 (in eng). 156. Roncagalli R, et al. (2005) Negative regulation of natural killer cell function by EAT2, a SAP-related adaptor. (Translated from eng) Nat Immunol 6(10):1002-1010 (in eng). 243 157. Dong Z, et al. (2009) Essential function for SAP family adaptors in the surveillance of hematopoietic cells by natural killer cells. (Translated from eng) Nat Immunol 10(9):973-980 (in eng). 158. Veillette A, Dong Z, Perez-Quintero LA, Zhong MC, & Cruz-Munoz ME (2009) Importance and mechanism of 'switch' function of SAP family adapters. (Translated from eng) Immunol Rev 232(1):229-239 (in eng). 159. Latour S, et al. (2003) Binding of SAP SH2 domain to FynT SH3 domain reveals a novel mechanism of receptor signalling in immune regulation. (Translated from eng) Nat Cell Biol 5(2):149-154 (in eng). 160. Nunez-Cruz S, et al. (2008) Differential requirement for the SAP-Fyn interaction during NK T cell development and function. (Translated from eng) J Immunol 181(4):2311-2320 (in eng). 161. Davidson D, et al. (2004) Genetic evidence linking SAP, the X-linked lymphoproliferative gene product, to Src-related kinase FynT in T(H)2 cytokine regulation. (Translated from eng) Immunity 21(5):707-717 (in eng). 162. Howie D, et al. (2002) The role of SAP in murine CD150 (SLAM)-mediated T-cell proliferation and interferon gamma production. (Translated from eng) Blood 100(8):2899-2907 (in eng). 163. Mikhalap SV, et al. (2004) The adaptor protein SH2D1A regulates signaling through CD150 (SLAM) in B cells. (Translated from eng) Blood 104(13):4063-4070 (in eng). 164. Cannons JL, et al. (2004) SAP regulates T(H)2 differentiation and PKC-thetamediated activation of NF-kappaB1. (Translated from eng) Immunity 21(5):693-706 (in eng). 165. Veillette A (2006) NK cell regulation by SLAM family receptors and SAP-related adapters. (Translated from eng) Immunological reviews 214:22-34 (in eng). 166. Wang N, et al. (2010) Cutting edge: The adapters EAT-2A and -2B are positive regulators of CD244- and CD84-dependent NK cell functions in the C57BL/6 mouse. (Translated from eng) J Immunol 185(10):5683-5687 (in eng). 167. Clarkson NG & Brown MH (2009) Inhibition and activation by CD244 depends on CD2 and phospholipase C-gamma1. (Translated from eng) J Biol Chem 284(37):24725-24734 (in eng). 168. Calpe S, et al. (2006) Identification and characterization of two related murine genes, Eat2a and Eat2b, encoding single SH2-domain adapters. (Translated from eng) Immunogenetics 58(1):15-25 (in eng). 244 169. Lasaro MO & Ertl HC (2009) New insights on adenovirus as vaccine vectors. (Translated from eng) Mol Ther 17(8):1333-1339 (in eng). 170. Russell WC (2009) Adenoviruses: update on structure and function. (Translated from eng) J Gen Virol 90(Pt 1):1-20 (in eng). 171. Russell WC (2000) Update on adenovirus and its vectors. (Translated from eng) J Gen Virol 81(Pt 11):2573-2604 (in eng). 172. Dhar S, et al. (2002) Flupirtine blocks apoptosis in batten patient lymphoblasts and in human postmitotic CLN3- and CLN2-deficient neurons. (Translated from eng) Ann Neurol 51(4):448-466 (in eng). 173. Amalfitano A, et al. (1998) Production and characterization of improved adenovirus vectors with the E1, E2b, and E3 genes deleted. (Translated from eng) J Virol 72(2):926-933 (in eng). 174. Engelhardt JF, Ye X, Doranz B, & Wilson JM (1994) Ablation of E2A in recombinant adenoviruses improves transgene persistence and decreases inflammatory response in mouse liver. (Translated from eng) Proc Natl Acad Sci U S A 91(13):6196-6200 (in eng). 175. Amalfitano A & Parks RJ (2002) Separating fact from fiction: assessing the potential of modified adenovirus vectors for use in human gene therapy. (Translated from eng) Curr Gene Ther 2(2):111-133 (in eng). 176. Raper SE, et al. (1998) Selective gene transfer into the liver of non-human primates with E1-deleted, E2A-defective, or E1-E4 deleted recombinant adenoviruses. (Translated from eng) Hum Gene Ther 9(5):671-679 (in eng). 177. Ding EY, et al. (2001) Long-term efficacy after [E1-, polymerase-] adenovirusmediated transfer of human acid-alpha-glucosidase gene into glycogen storage disease type II knockout mice. (Translated from eng) Hum Gene Ther 12(8):955-965 (in eng). 178. Howe SJ, et al. (2008) Insertional mutagenesis combined with acquired somatic mutations causes leukemogenesis following gene therapy of SCID-X1 patients. (Translated from eng) J Clin Invest 118(9):3143-3150 (in eng). 179. Hartman ZC, Appledorn DM, & Amalfitano A (2008) Adenovirus vector induced innate immune responses: impact upon efficacy and toxicity in gene therapy and vaccine applications. (Translated from eng) Virus Res 132(1-2):1-14 (in eng). 180. Rhee EG, et al. (2011) Multiple innate immune pathways contribute to the immunogenicity of recombinant adenovirus vaccine vectors. (Translated from eng) J Virol 85(1):315-323 (in eng). 245 181. Hartman ZC, et al. (2007) Adenovirus infection triggers a rapid, MyD88-regulated transcriptome response critical to acute-phase and adaptive immune responses in vivo. (Translated from eng) J Virol 81(4):1796-1812 (in eng). 182. Seregin SS & Amalfitano A (2010) Improving adenovirus based gene transfer: strategies to accomplish immune evasion. (Translated from eng) Viruses 2(9):20132036 (in eng). 183. Appledorn DM, et al. (2008) Complex interactions with several arms of the complement system dictate innate and humoral immunity to adenoviral vectors. (Translated from eng) Gene Ther 15(24):1606-1617 (in eng). 184. Appledorn DM, et al. (2008) Adenovirus vector-induced innate inflammatory mediators, MAPK signaling, as well as adaptive immune responses are dependent upon both TLR2 and TLR9 in vivo. (Translated from eng) J Immunol 181(3):21342144 (in eng). 185. Basner-Tschakarjan E, et al. (2006) Adenovirus efficiently transduces plasmacytoid dendritic cells resulting in TLR9-dependent maturation and IFN-alpha production. (Translated from eng) J Gene Med 8(11):1300-1306 (in eng). 186. Schnell MA, et al. (2001) Activation of innate immunity in nonhuman primates following intraportal administration of adenoviral vectors. (Translated from eng) Mol Ther 3(5 Pt 1):708-722 (in eng). 187. Di Paolo NC, et al. (2009) Virus binding to a plasma membrane receptor triggers interleukin-1 alpha-mediated proinflammatory macrophage response in vivo. (Translated from eng) Immunity 31(1):110-121 (in eng). 188. Shifrin AL, Chirmule N, Zhang Y, & Raper SE (2005) Macrophage ablation attenuates adenoviral vector-induced pancreatitis. (Translated from eng) Surgery 137(5):545551 (in eng). 189. Lore K, et al. (2007) Myeloid and plasmacytoid dendritic cells are susceptible to recombinant adenovirus vectors and stimulate polyfunctional memory T cell responses. (Translated from eng) J Immunol 179(3):1721-1729 (in eng). 190. Lindsay RW, et al. (2010) CD8+ T cell responses following replication-defective adenovirus serotype 5 immunization are dependent on CD11c+ dendritic cells but show redundancy in their requirement of TLR and nucleotide-binding oligomerization domain-like receptor signaling. (Translated from eng) J Immunol 185(3):1513-1521 (in eng). 191. Aldhamen YA, et al. (2011) Expression of the SLAM family of receptors adapter EAT2 as a novel strategy for enhancing beneficial immune responses to vaccine antigens. (Translated from eng) J Immunol 186(2):722-732 (in eng). 246 192. Schroers R, et al. (2004) Gene transfer into human T lymphocytes and natural killer cells by Ad5/F35 chimeric adenoviral vectors. (Translated from eng) Exp Hematol 32(6):536-546 (in eng). 193. Zhu J, Huang X, & Yang Y (2008) A critical role for type I IFN-dependent NK cell activation in innate immune elimination of adenoviral vectors in vivo. (Translated from eng) Molecular therapy : the journal of the American Society of Gene Therapy 16(7):1300-1307 (in eng). 194. Hensley SE, Giles-Davis W, McCoy KC, Weninger W, & Ertl HC (2005) Dendritic cell maturation, but not CD8+ T cell induction, is dependent on type I IFN signaling during vaccination with adenovirus vectors. (Translated from eng) J Immunol 175(9):6032-6041 (in eng). 195. Zhu J, Huang X, & Yang Y (2007) Innate immune response to adenoviral vectors is mediated by both Toll-like receptor-dependent and -independent pathways. (Translated from eng) J Virol 81(7):3170-3180 (in eng). 196. Huarte E, et al. (2006) Recombinant adenoviral vectors turn on the type I interferon system without inhibition of transgene expression and viral replication. (Translated from eng) Mol Ther 14(1):129-138 (in eng). 197. Wickham TJ, Mathias P, Cheresh DA, & Nemerow GR (1993) Integrins alpha v beta 3 and alpha v beta 5 promote adenovirus internalization but not virus attachment. (Translated from eng) Cell 73(2):309-319 (in eng). 198. Bergelson JM, et al. (1997) Isolation of a common receptor for Coxsackie B viruses and adenoviruses 2 and 5. (Translated from eng) Science 275(5304):1320-1323 (in eng). 199. Tibbles LA, et al. (2002) Activation of p38 and ERK signaling during adenovirus vector cell entry lead to expression of the C-X-C chemokine IP-10. (Translated from eng) J Virol 76(4):1559-1568 (in eng). 200. Nociari M, Ocheretina O, Schoggins JW, & Falck-Pedersen E (2007) Sensing infection by adenovirus: Toll-like receptor-independent viral DNA recognition signals activation of the interferon regulatory factor 3 master regulator. (Translated from eng) J Virol 81(8):4145-4157 (in eng). 201. Shayakhmetov DM, Di Paolo NC, & Mossman KL (2010) Recognition of virus infection and innate host responses to viral gene therapy vectors. (Translated from eng) Mol Ther 18(8):1422-1429 (in eng). 202. Takaoka A, et al. (2007) DAI (DLM-1/ZBP1) is a cytosolic DNA sensor and an activator of innate immune response. (Translated from eng) Nature 448(7152):501505 (in eng). 247 203. Ishii KJ & Akira S (2006) Innate immune recognition of, and regulation by, DNA. (Translated from eng) Trends Immunol 27(11):525-532 (in eng). 204. Muruve DA, et al. (2008) The inflammasome recognizes cytosolic microbial and host DNA and triggers an innate immune response. (Translated from eng) Nature 452(7183):103-107 (in eng). 205. Barlan AU, Griffin TM, McGuire KA, & Wiethoff CM (2011) Adenovirus membrane penetration activates the NLRP3 inflammasome. (Translated from eng) J Virol 85(1):146-155 (in eng). 206. Lotze MT & Tracey KJ (2005) High-mobility group box 1 protein (HMGB1): nuclear weapon in the immune arsenal. (Translated from eng) Nat Rev Immunol 5(4):331342 (in eng). 207. Minamitani T, Iwakiri D, & Takada K (2011) Adenovirus virus-associated RNAs induce type I interferon expression through a RIG-I-mediated pathway. (Translated from eng) J Virol 85(8):4035-4040 (in eng). 208. Barouch DH (2010) Novel adenovirus vector-based vaccines for HIV-1. (Translated from eng) Curr Opin HIV AIDS 5(5):386-390 (in eng). 209. Priddy FH, et al. (2008) Safety and immunogenicity of a replication-incompetent adenovirus type 5 HIV-1 clade B gag/pol/nef vaccine in healthy adults. (Translated from eng) Clin Infect Dis 46(11):1769-1781 (in eng). 210. Buchbinder SP, et al. (2008) Efficacy assessment of a cell-mediated immunity HIV-1 vaccine (the Step Study): a double-blind, randomised, placebo-controlled, test-ofconcept trial. (Translated from eng) Lancet 372(9653):1881-1893 (in eng). 211. Gabitzsch ES, et al. (2009) A preliminary and comparative evaluation of a novel Ad5 [E1-, E2b-] recombinant-based vaccine used to induce cell mediated immune responses. (Translated from eng) Immunol Lett 122(1):44-51 (in eng). 212. Gabitzsch ES, et al. (2010) Anti-tumor immunotherapy despite immunity to adenovirus using a novel adenoviral vector Ad5 [E1-, E2b-]-CEA. (Translated from eng) Cancer Immunol Immunother 59(7):1131-1135 (in eng). 213. Gabitzsch ES, Xu Y, Balcaitis S, Balint JP, Jr., & Jones FR (2011) An Ad5[E1-, E2b-]HER2/neu vector induces immune responses and inhibits HER2/neu expressing tumor progression in Ad5 immune mice. (Translated from eng) Cancer Gene Ther 18(5):326-335 (in eng). 214. Gabitzsch ES, et al. (2009) Novel Adenovirus type 5 vaccine platform induces cellular immunity against HIV-1 Gag, Pol, Nef despite the presence of Ad5 immunity. (Translated from eng) Vaccine 27(46):6394-6398 (in eng). 248 215. Osada T, et al. (2009) Optimization of vaccine responses with an E1, E2b and E3deleted Ad5 vector circumvents pre-existing anti-vector immunity. (Translated from eng) Cancer Gene Ther 16(9):673-682 (in eng). 216. Rerks-Ngarm S, et al. (2009) Vaccination with ALVAC and AIDSVAX to Prevent HIV-1 Infection in Thailand. (Translated from Eng) The New England journal of medicine (in Eng). 217. McElrath MJ, et al. (2008) HIV-1 vaccine-induced immunity in the test-of-concept Step Study: a case-cohort analysis. (Translated from eng) Lancet 372(9653):18941905 (in eng). 218. Kool M, et al. (2008) Cutting edge: alum adjuvant stimulates inflammatory dendritic cells through activation of the NALP3 inflammasome. (Translated from eng) J Immunol 181(6):3755-3759 (in eng). 219. Mbow ML, De Gregorio E, Valiante NM, & Rappuoli R (New adjuvants for human vaccines. (Translated from Eng) Current opinion in immunology (in Eng). 220. Wille-Reece U, et al. (2005) HIV Gag protein conjugated to a Toll-like receptor 7/8 agonist improves the magnitude and quality of Th1 and CD8+ T cell responses in nonhuman primates. (Translated from eng) Proceedings of the National Academy of Sciences of the United States of America 102(42):15190-15194 (in eng). 221. Hartman ZC, et al. (Ligand-independent toll-like receptor signals generated by ectopic overexpression of MyD88 generate local and systemic antitumor immunity. (Translated from eng) Cancer Res 70(18):7209-7220 (in eng). 222. Rerks-Ngarm S, et al. (2010) Defining the objectives of the AIDS vaccine for Asia network: report of the WHO-UNAIDS/Global HIV vaccine enterprise regional consultation on expanding AIDS vaccine research and development capacity in Asia. (Translated from eng) Curr Opin HIV AIDS 5(5):435-452 (in eng). 223. Hahn BH, Shaw GM, De Cock KM, & Sharp PM (2000) AIDS as a zoonosis: scientific and public health implications. (Translated from eng) Science 287(5453):607-614 (in eng). 224. Hemelaar J (2012) The origin and diversity of the HIV-1 pandemic. (Translated from Eng) Trends Mol Med (in Eng). 225. Roberts JD, Bebenek K, & Kunkel TA (1988) The accuracy of reverse transcriptase from HIV-1. (Translated from eng) Science 242(4882):1171-1173 (in eng). 226. Korber B, et al. (2001) Evolutionary and immunological implications of contemporary HIV-1 variation. (Translated from eng) Br Med Bull 58:19-42 (in eng). 249 227. Rousseau CM, et al. (2007) Extensive intrasubtype recombination in South African human immunodeficiency virus type 1 subtype C infections. (Translated from eng) J Virol 81(9):4492-4500 (in eng). 228. Peeters M, et al. (1999) Characterization of a highly replicative intergroup M/O human immunodeficiency virus type 1 recombinant isolated from a Cameroonian patient. (Translated from eng) J Virol 73(9):7368-7375 (in eng). 229. Hemelaar J, Gouws E, Ghys PD, & Osmanov S (2011) Global trends in molecular epidemiology of HIV-1 during 2000-2007. (Translated from eng) Aids 25(5):679689 (in eng). 230. Pope M & Haase AT (2003) Transmission, acute HIV-1 infection and the quest for strategies to prevent infection. (Translated from eng) Nat Med 9(7):847-852 (in eng). 231. Tomaras GD & Haynes BF (2009) HIV-1-specific antibody responses during acute and chronic HIV-1 infection. (Translated from eng) Curr Opin HIV AIDS 4(5):373-379 (in eng). 232. McMichael AJ, Borrow P, Tomaras GD, Goonetilleke N, & Haynes BF (2010) The immune response during acute HIV-1 infection: clues for vaccine development. (Translated from eng) Nat Rev Immunol 10(1):11-23 (in eng). 233. Lehner T, et al. (2008) The emerging role of innate immunity in protection against HIV-1 infection. (Translated from eng) Vaccine 26(24):2997-3001 (in eng). 234. Hussain LA & Lehner T (1995) Comparative investigation of Langerhans' cells and potential receptors for HIV in oral, genitourinary and rectal epithelia. (Translated from eng) Immunology 85(3):475-484 (in eng). 235. Lehner T, Wang Y, Whittall T, & Seidl T (2011) Innate immunity and HIV-1 infection. (Translated from eng) Adv Dent Res 23(1):19-22 (in eng). 236. Altfeld M, Fadda L, Frleta D, & Bhardwaj N (2011) DCs and NK cells: critical effectors in the immune response to HIV-1. (Translated from eng) Nat Rev Immunol 11(3):176-186 (in eng). 237. Donaghy H, Gazzard B, Gotch F, & Patterson S (2003) Dysfunction and infection of freshly isolated blood myeloid and plasmacytoid dendritic cells in patients infected with HIV-1. (Translated from eng) Blood 101(11):4505-4511 (in eng). 238. Wu L & KewalRamani VN (2006) Dendritic-cell interactions with HIV: infection and viral dissemination. (Translated from eng) Nat Rev Immunol 6(11):859-868 (in eng). 250 239. Romagnani C, et al. (2005) Activation of human NK cells by plasmacytoid dendritic cells and its modulation by CD4+ T helper cells and CD4+ CD25hi T regulatory cells. (Translated from eng) Eur J Immunol 35(8):2452-2458 (in eng). 240. Blanchet FP, et al. (2010) Human immunodeficiency virus-1 inhibition of immunoamphisomes in dendritic cells impairs early innate and adaptive immune responses. (Translated from eng) Immunity 32(5):654-669 (in eng). 241. Herbeuval JP, et al. (2005) Regulation of TNF-related apoptosis-inducing ligand on primary CD4+ T cells by HIV-1: role of type I IFN-producing plasmacytoid dendritic cells. (Translated from eng) Proc Natl Acad Sci U S A 102(39):13974-13979 (in eng). 242. Manches O, et al. (2008) HIV-activated human plasmacytoid DCs induce Tregs through an indoleamine 2,3-dioxygenase-dependent mechanism. (Translated from eng) J Clin Invest 118(10):3431-3439 (in eng). 243. Lanier LL (2008) Evolutionary struggles between NK cells and viruses. (Translated from eng) Nat Rev Immunol 8(4):259-268 (in eng). 244. Mailliard RB, et al. (2003) Dendritic cells mediate NK cell help for Th1 and CTL responses: two-signal requirement for the induction of NK cell helper function. (Translated from eng) J Immunol 171(5):2366-2373 (in eng). 245. Lanier LL (2008) Up on the tightrope: natural killer cell activation and inhibition. (Translated from eng) Nat Immunol 9(5):495-502 (in eng). 246. Collins KL, Chen BK, Kalams SA, Walker BD, & Baltimore D (1998) HIV-1 Nef protein protects infected primary cells against killing by cytotoxic T lymphocytes. (Translated from eng) Nature 391(6665):397-401 (in eng). 247. Bonaparte MI & Barker E (2004) Killing of human immunodeficiency virus-infected primary T-cell blasts by autologous natural killer cells is dependent on the ability of the virus to alter the expression of major histocompatibility complex class I molecules. (Translated from eng) Blood 104(7):2087-2094 (in eng). 248. Cerboni C, et al. (2007) Human immunodeficiency virus 1 Nef protein downmodulates the ligands of the activating receptor NKG2D and inhibits natural killer cell-mediated cytotoxicity. (Translated from eng) J Gen Virol 88(Pt 1):242-250 (in eng). 249. Stacey AR, et al. (2009) Induction of a striking systemic cytokine cascade prior to peak viremia in acute human immunodeficiency virus type 1 infection, in contrast to more modest and delayed responses in acute hepatitis B and C virus infections. (Translated from eng) J Virol 83(8):3719-3733 (in eng). 251 250. Lehner T, et al. (2000) Up-regulation of beta-chemokines and down-modulation of CCR5 co-receptors inhibit simian immunodeficiency virus transmission in nonhuman primates. (Translated from eng) Immunology 99(4):569-577 (in eng). 251. Sheehy AM, Gaddis NC, Choi JD, & Malim MH (2002) Isolation of a human gene that inhibits HIV-1 infection and is suppressed by the viral Vif protein. (Translated from eng) Nature 418(6898):646-650 (in eng). 252. Chen K, et al. (2006) Alpha interferon potently enhances the anti-human immunodeficiency virus type 1 activity of APOBEC3G in resting primary CD4 T cells. (Translated from eng) J Virol 80(15):7645-7657 (in eng). 253. Luo K, et al. (2007) Cytidine deaminases APOBEC3G and APOBEC3F interact with human immunodeficiency virus type 1 integrase and inhibit proviral DNA formation. (Translated from eng) J Virol 81(13):7238-7248 (in eng). 254. Pido-Lopez J, et al. (2007) Stimulation of cell surface CCR5 and CD40 molecules by their ligands or by HSP70 up-regulates APOBEC3G expression in CD4(+) T cells and dendritic cells. (Translated from eng) J Immunol 178(3):1671-1679 (in eng). 255. Wang Y, et al. (2009) Mucosal immunization in macaques upregulates the innate APOBEC 3G anti-viral factor in CD4(+) memory T cells. (Translated from eng) Vaccine 27(6):870-881 (in eng). 256. Nisole S, Stoye JP, & Saib A (2005) TRIM family proteins: retroviral restriction and antiviral defence. (Translated from eng) Nat Rev Microbiol 3(10):799-808 (in eng). 257. Neil SJ, Zang T, & Bieniasz PD (2008) Tetherin inhibits retrovirus release and is antagonized by HIV-1 Vpu. (Translated from eng) Nature 451(7177):425-430 (in eng). 258. Okumura A, et al. (2008) HIV-1 accessory proteins VPR and Vif modulate antiviral response by targeting IRF-3 for degradation. (Translated from eng) Virology 373(1):85-97 (in eng). 259. Solis M, et al. (2011) RIG-I-mediated antiviral signaling is inhibited in HIV-1 infection by a protease-mediated sequestration of RIG-I. (Translated from eng) J Virol 85(3):1224-1236 (in eng). 260. Mehandru S, et al. (2004) Primary HIV-1 infection is associated with preferential depletion of CD4+ T lymphocytes from effector sites in the gastrointestinal tract. (Translated from eng) J Exp Med 200(6):761-770 (in eng). 261. Goonetilleke N, et al. (2009) The first T cell response to transmitted/founder virus contributes to the control of acute viremia in HIV-1 infection. (Translated from eng) J Exp Med 206(6):1253-1272 (in eng). 252 262. Kiepiela P, et al. (2007) CD8+ T-cell responses to different HIV proteins have discordant associations with viral load. (Translated from eng) Nat Med 13(1):46-53 (in eng). 263. Fellay J, et al. (2007) A whole-genome association study of major determinants for host control of HIV-1. (Translated from eng) Science 317(5840):944-947 (in eng). 264. Matthews PC, et al. (2009) HLA footprints on human immunodeficiency virus type 1 are associated with interclade polymorphisms and intraclade phylogenetic clustering. (Translated from eng) J Virol 83(9):4605-4615 (in eng). 265. Schmitz JE, et al. (1999) Control of viremia in simian immunodeficiency virus infection by CD8+ lymphocytes. (Translated from eng) Science 283(5403):857-860 (in eng). 266. Hansen SG, et al. (2011) Profound early control of highly pathogenic SIV by an effector memory T-cell vaccine. (Translated from eng) Nature 473(7348):523-527 (in eng). 267. Barouch DH, et al. (2012) Vaccine protection against acquisition of neutralizationresistant SIV challenges in rhesus monkeys. (Translated from Eng) Nature (in Eng). 268. Picker LJ, Hansen SG, & Lifson JD (2012) New Paradigms for HIV/AIDS Vaccine Development. (Translated from eng) Annu Rev Med 63:95-111 (in eng). 269. Rerks-Ngarm S, et al. (2009) Vaccination with ALVAC and AIDSVAX to prevent HIV-1 infection in Thailand. (Translated from eng) N Engl J Med 361(23):2209-2220 (in eng). 270. Tomaras GD, et al. (2008) Initial B-cell responses to transmitted human immunodeficiency virus type 1: virion-binding immunoglobulin M (IgM) and IgG antibodies followed by plasma anti-gp41 antibodies with ineffective control of initial viremia. (Translated from eng) J Virol 82(24):12449-12463 (in eng). 271. Binley JM, et al. (2008) Profiling the specificity of neutralizing antibodies in a large panel of plasmas from patients chronically infected with human immunodeficiency virus type 1 subtypes B and C. (Translated from eng) J Virol 82(23):11651-11668 (in eng). 272. Wei X, et al. (2003) Antibody neutralization and escape by HIV-1. (Translated from eng) Nature 422(6929):307-312 (in eng). 273. Wyatt R, et al. (1998) The antigenic structure of the HIV gp120 envelope glycoprotein. (Translated from eng) Nature 393(6686):705-711 (in eng). 253 274. Hirbod T, et al. (2008) HIV-neutralizing immunoglobulin A and HIV-specific proliferation are independently associated with reduced HIV acquisition in Kenyan sex workers. (Translated from eng) Aids 22(6):727-735 (in eng). 275. Veazey RS, et al. (2003) Prevention of virus transmission to macaque monkeys by a vaginally applied monoclonal antibody to HIV-1 gp120. (Translated from eng) Nat Med 9(3):343-346 (in eng). 276. Alter G & Moody MA (2010) The humoral response to HIV-1: new insights, renewed focus. (Translated from eng) J Infect Dis 202 Suppl 2:S315-322 (in eng). 277. Connor RI, et al. (1998) Immunological and virological analyses of persons infected by human immunodeficiency virus type 1 while participating in trials of recombinant gp120 subunit vaccines. (Translated from eng) J Virol 72(2):15521576 (in eng). 278. Hoxie JA (2010) Toward an antibody-based HIV-1 vaccine. (Translated from eng) Annu Rev Med 61:135-152 (in eng). 279. Flynn NM, et al. (2005) Placebo-controlled phase 3 trial of a recombinant glycoprotein 120 vaccine to prevent HIV-1 infection. (Translated from eng) J Infect Dis 191(5):654-665 (in eng). 280. Robinson HL & Amara RR (2005) T cell vaccines for microbial infections. (Translated from eng) Nat Med 11(4 Suppl):S25-32 (in eng). 281. Pontesilli O, et al. (1998) Longitudinal analysis of human immunodeficiency virus type 1-specific cytotoxic T lymphocyte responses: a predominant gag-specific response is associated with nonprogressive infection. (Translated from eng) J Infect Dis 178(4):1008-1018 (in eng). 282. Seregin SS & Amalfitano A (2009) Overcoming pre-existing adenovirus immunity by genetic engineering of adenovirus-based vectors. (Translated from eng) Expert Opin Biol Ther 9(12):1521-1531 (in eng). 283. Hutnick NA, et al. (2009) Baseline Ad5 serostatus does not predict Ad5 HIV vaccineinduced expansion of adenovirus-specific CD4+ T cells. (Translated from eng) Nat Med 15(8):876-878 (in eng). 284. O'Brien KL, et al. (2009) Adenovirus-specific immunity after immunization with an Ad5 HIV-1 vaccine candidate in humans. (Translated from eng) Nat Med 15(8):873875 (in eng). 285. Letvin NL, et al. (2011) Immune and Genetic Correlates of Vaccine Protection Against Mucosal Infection by SIV in Monkeys. (Translated from eng) Sci Transl Med 3(81):81ra36 (in eng). 254 286. Veillette A, Dong Z, Perez-Quintero LA, Zhong MC, & Cruz-Munoz ME (2009) Importance and mechanism of 'switch' function of SAP family adapters. (Translated from eng) Immunological reviews 232(1):229-239 (in eng). 287. Veillette A, Dong Z, & Latour S (2007) Consequence of the SLAM-SAP signaling pathway in innate-like and conventional lymphocytes. (Translated from eng) Immunity 27(5):698-710 (in eng). 288. Flaig RM, Stark S, & Watzl C (2004) Cutting edge: NTB-A activates NK cells via homophilic interaction. (Translated from eng) J Immunol 172(11):6524-6527 (in eng). 289. Ma CS, Nichols KE, & Tangye SG (2007) Regulation of cellular and humoral immune responses by the SLAM and SAP families of molecules. (Translated from eng) Annual review of immunology 25:337-379 (in eng). 290. Calpe S, et al. (2008) The SLAM and SAP gene families control innate and adaptive immune responses. (Translated from eng) Advances in immunology 97:177-250 (in eng). 291. Bleharski JR, Niazi KR, Sieling PA, Cheng G, & Modlin RL (2001) Signaling lymphocytic activation molecule is expressed on CD40 ligand-activated dendritic cells and directly augments production of inflammatory cytokines. (Translated from eng) J Immunol 167(6):3174-3181 (in eng). 292. Wang N, et al. (2004) The cell surface receptor SLAM controls T cell and macrophage functions. (Translated from eng) J Exp Med 199(9):1255-1264 (in eng). 293. Takeshita F, et al. (2006) Toll-like receptor adaptor molecules enhance DNA-raised adaptive immune responses against influenza and tumors through activation of innate immunity. (Translated from eng) Journal of virology 80(13):6218-6224 (in eng). 294. Hartman ZC, et al. (2007) Adenovirus infection triggers a rapid, MyD88-regulated transcriptome response critical to acute-phase and adaptive immune responses in vivo. (Translated from eng) Journal of virology 81(4):1796-1812 (in eng). 295. Zhu J, Huang X, & Yang Y (2008) A critical role for type I IFN-dependent NK cell activation in innate immune elimination of adenoviral vectors in vivo. (Translated from eng) Mol Ther 16(7):1300-1307 (in eng). 296. Muruve DA (2004) The innate immune response to adenovirus vectors. (Translated from eng) Human gene therapy 15(12):1157-1166 (in eng). 297. Gonzalez-Aseguinolaza G, et al. (2002) Natural killer T cell ligand alphagalactosylceramide enhances protective immunity induced by malaria vaccines. 255 (Translated from eng) The Journal of experimental medicine 195(5):617-624 (in eng). 298. Betts MR, et al. (2006) HIV nonprogressors preferentially maintain highly functional HIV-specific CD8+ T cells. (Translated from eng) Blood 107(12):4781-4789 (in eng). 299. Darrah PA, et al. (2007) Multifunctional TH1 cells define a correlate of vaccinemediated protection against Leishmania major. (Translated from eng) Nat Med 13(7):843-850 (in eng). 300. Yang Y, Huang CT, Huang X, & Pardoll DM (2004) Persistent Toll-like receptor signals are required for reversal of regulatory T cell-mediated CD8 tolerance. (Translated from eng) Nature immunology 5(5):508-515 (in eng). 301. Veillette A (2006) Immune regulation by SLAM family receptors and SAP-related adaptors. (Translated from eng) Nature reviews 6(1):56-66 (in eng). 302. Vitale M, et al. (2005) NK-dependent DC maturation is mediated by TNFalpha and IFNgamma released upon engagement of the NKp30 triggering receptor. (Translated from eng) Blood 106(2):566-571 (in eng). 303. Mailliard RB, et al. (2003) Dendritic cells mediate NK cell help for Th1 and CTL responses: two-signal requirement for the induction of NK cell helper function. (Translated from eng) J Immunol 171(5):2366-2373 (in eng). 304. Kelly JM, et al. (2002) Induction of tumor-specific T cell memory by NK cellmediated tumor rejection. (Translated from eng) Nature immunology 3(1):83-90 (in eng). 305. Tassi I & Colonna M (2005) The cytotoxicity receptor CRACC (CS-1) recruits EAT-2 and activates the PI3K and phospholipase Cgamma signaling pathways in human NK cells. (Translated from eng) J Immunol 175(12):7996-8002 (in eng). 306. Eissmann P & Watzl C (2006) Molecular analysis of NTB-A signaling: a role for EAT2 in NTB-A-mediated activation of human NK cells. (Translated from eng) J Immunol 177(5):3170-3177 (in eng). 307. Ostrakhovitch EA, Wang Y, & Li SS (2009) SAP binds to CD22 and regulates B cell inhibitory signaling and calcium flux. (Translated from eng) Cell Signal 21(4):540550 (in eng). 308. Li C, et al. (2008) The X-linked lymphoproliferative syndrome gene product SAP regulates B cell function through the FcgammaRIIB receptor. (Translated from eng) Cell Signal 20(11):1960-1967 (in eng). 256 309. Liu J, et al. (2009) Immune control of an SIV challenge by a T-cell-based vaccine in rhesus monkeys. (Translated from eng) Nature 457(7225):87-91 (in eng). 310. Kong WP, et al. (2009) Expanded breadth of the T-cell response to mosaic human immunodeficiency virus type 1 envelope DNA vaccination. (Translated from eng) Journal of virology 83(5):2201-2215 (in eng). 311. Hafalla JC, Silvie O, & Matuschewski K (2011) Cell biology and immunology of malaria. (Translated from eng) Immunol Rev 240(1):297-316 (in eng). 312. Anonymous (2008) World Malaria Report 2009. (World Health Organization, Geneva), p 190. 313. Poespoprodjo JR, et al. (2009) Vivax malaria: a major cause of morbidity in early infancy. (Translated from eng) Clin Infect Dis 48(12):1704-1712 (in eng). 314. Good MF & Doolan DL (2010) Malaria vaccine design: immunological considerations. (Translated from eng) Immunity 33(4):555-566 (in eng). 315. Artavanis-Tsakonas K & Riley EM (2002) Innate immune response to malaria: rapid induction of IFN-gamma from human NK cells by live Plasmodium falciparuminfected erythrocytes. (Translated from eng) J Immunol 169(6):2956-2963 (in eng). 316. Pichyangkul S, et al. (2004) Malaria blood stage parasites activate human plasmacytoid dendritic cells and murine dendritic cells through a Toll-like receptor 9-dependent pathway. (Translated from eng) J Immunol 172(8):4926-4933 (in eng). 317. Krishnegowda G, et al. (2005) Induction of proinflammatory responses in macrophages by the glycosylphosphatidylinositols of Plasmodium falciparum: cell signaling receptors, glycosylphosphatidylinositol (GPI) structural requirement, and regulation of GPI activity. (Translated from eng) J Biol Chem 280(9):8606-8616 (in eng). 318. Doolan DL & Martinez-Alier N (2006) Immune response to pre-erythrocytic stages of malaria parasites. (Translated from eng) Curr Mol Med 6(2):169-185 (in eng). 319. Tsuji M (2010) A retrospective evaluation of the role of T cells in the development of malaria vaccine. (Translated from eng) Exp Parasitol 126(3):421-425 (in eng). 320. Cohen J, Nussenzweig V, Nussenzweig R, Vekemans J, & Leach A (2010) From the circumsporozoite protein to the RTS, S/AS candidate vaccine. (Translated from eng) Hum Vaccin 6(1):90-96 (in eng). 321. Weiss WR, Sedegah M, Beaudoin RL, Miller LH, & Good MF (1988) CD8+ T cells (cytotoxic/suppressors) are required for protection in mice immunized with 257 malaria sporozoites. (Translated from eng) Proc Natl Acad Sci U S A 85(2):573-576 (in eng). 322. Chakravarty S, et al. (2007) CD8+ T lymphocytes protective against malaria liver stages are primed in skin-draining lymph nodes. (Translated from eng) Nat Med 13(9):1035-1041 (in eng). 323. Hafalla JC, Cockburn IA, & Zavala F (2006) Protective and pathogenic roles of CD8+ T cells during malaria infection. (Translated from eng) Parasite Immunol 28(12):15-24 (in eng). 324. Oliveira GA, et al. (2008) Class II-restricted protective immunity induced by malaria sporozoites. (Translated from eng) Infect Immun 76(3):1200-1206 (in eng). 325. Jobe O, et al. (2007) Genetically attenuated Plasmodium berghei liver stages induce sterile protracted protection that is mediated by major histocompatibility complex Class I-dependent interferon-gamma-producing CD8+ T cells. (Translated from eng) J Infect Dis 196(4):599-607 (in eng). 326. Tsuji M, et al. (1994) Gamma delta T cells contribute to immunity against the liver stages of malaria in alpha beta T-cell-deficient mice. (Translated from eng) Proc Natl Acad Sci U S A 91(1):345-349 (in eng). 327. Walther M, et al. (2005) Upregulation of TGF-beta, FOXP3, and CD4+CD25+ regulatory T cells correlates with more rapid parasite growth in human malaria infection. (Translated from eng) Immunity 23(3):287-296 (in eng). 328. Dorfman JR, et al. (2005) B cell memory to 3 Plasmodium falciparum blood-stage antigens in a malaria-endemic area. (Translated from eng) J Infect Dis 191(10):1623-1630 (in eng). 329. Kemp K, et al. (2002) Acute P. falciparum malaria induces a loss of CD28- T IFNgamma producing cells. (Translated from eng) Parasite Immunol 24(11-12):545-548 (in eng). 330. Sponaas AM, et al. (2006) Malaria infection changes the ability of splenic dendritic cell populations to stimulate antigen-specific T cells. (Translated from eng) J Exp Med 203(6):1427-1433 (in eng). 331. Barry AE, Schultz L, Buckee CO, & Reeder JC (2009) Contrasting population structures of the genes encoding ten leading vaccine-candidate antigens of the human malaria parasite, Plasmodium falciparum. (Translated from eng) PLoS One 4(12):e8497 (in eng). 258 332. Butler NS, et al. (2012) Therapeutic blockade of PD-L1 and LAG-3 rapidly clears established blood-stage Plasmodium infection. (Translated from eng) Nat Immunol 13(2):188-195 (in eng). 333. Hoffman SL, et al. (2002) Protection of humans against malaria by immunization with radiation-attenuated Plasmodium falciparum sporozoites. (Translated from eng) J Infect Dis 185(8):1155-1164 (in eng). 334. Baruch DI, et al. (1995) Cloning the P. falciparum gene encoding PfEMP1, a malarial variant antigen and adherence receptor on the surface of parasitized human erythrocytes. (Translated from eng) Cell 82(1):77-87 (in eng). 335. Plassmeyer ML, et al. (2009) Structure of the Plasmodium falciparum circumsporozoite protein, a leading malaria vaccine candidate. (Translated from eng) J Biol Chem 284(39):26951-26963 (in eng). 336. Hamilton AJ, Suhrbier A, Nicholas J, & Sinden RE (1988) Immunoelectron microscopic localization of circumsporozoite antigen in the differentiating exoerythrocytic trophozoite of Plasmodium berghei. (Translated from eng) Cell Biol Int Rep 12(2):123-129 (in eng). 337. Overstreet MG, Cockburn IA, Chen YC, & Zavala F (2008) Protective CD8 T cells against Plasmodium liver stages: immunobiology of an 'unnatural' immune response. (Translated from eng) Immunol Rev 225:272-283 (in eng). 338. Romero P, et al. (1989) Cloned cytotoxic T cells recognize an epitope in the circumsporozoite protein and protect against malaria. (Translated from eng) Nature 341(6240):323-326 (in eng). 339. Sadoff JC, et al. (1988) Oral Salmonella typhimurium vaccine expressing circumsporozoite protein protects against malaria. (Translated from eng) Science 240(4850):336-338 (in eng). 340. Rodrigues EG, Zavala F, Eichinger D, Wilson JM, & Tsuji M (1997) Single immunizing dose of recombinant adenovirus efficiently induces CD8+ T cell-mediated protective immunity against malaria. J Immunol 158(3):1268-1274. 341. Gordon DM, et al. (1995) Safety, Immunogenicity, and Efficacy of a Recombinantly Produced Plasmodium falciparum Circumsporozoite Protein-Hepatitis B Surface Antigen Subunit Vaccine. The Journal of Infectious Diseases 171(6):1576-1585. 342. Kester Kent E, et al. (2009) Randomized, Double―Blind, Phase 2a Trial of Falciparum Malaria Vaccines RTS,S/AS01B and RTS,S/AS02A in Malaria―Naive Adults: Safety, Efficacy, and Immunologic Associates of Protection. The Journal of Infectious Diseases 200(3):337-346. 259 343. Bejon P, et al. (2008) Efficacy of RTS,S/AS01E Vaccine against Malaria in Children 5 to 17 Months of Age. N Engl J Med 359(24):2521-2532. 344. Gregson AL, et al. (2008) Phase I Trial of an Alhydrogel Adjuvanted Hepatitis B Core Virus-Like Particle Containing Epitopes of Plasmodium falciparum Circumsporozoite Protein. PLoS ONE 3(2):e1556. 345. Kester KE, et al. (2008) Phase 2a trial of 0, 1, and 3 month and 0, 7, and 28 day immunization schedules of malaria vaccine RTS,S/AS02 in malaria-naïve adults at the Walter Reed Army Institute of Research. Vaccine 26(18):2191-2202. 346. Casares S, Brumeanu TD, & Richie TL (2010) The RTS,S malaria vaccine. (Translated from eng) Vaccine 28(31):4880-4894 (in eng). 347. Sun P, et al. (2003) Protective Immunity Induced with Malaria Vaccine, RTS,S, Is Linked to Plasmodium falciparum Circumsporozoite Protein-Specific CD4+ and CD8+ T Cells Producing IFN-{gamma}. J Immunol 171(12):6961-6967. 348. Ophorst OJAE, et al. (2006) Immunogenicity and Protection of a Recombinant Human Adenovirus Serotype 35-Based Malaria Vaccine against Plasmodium yoelii in Mice. Infect. Immun. 74(1):313-320. 349. Shott JP, et al. (2008) Adenovirus 5 and 35 vectors expressing Plasmodium falciparum circumsporozoite surface protein elicit potent antigen-specific cellular IFN-[gamma] and antibody responses in mice. Vaccine 26(23):2818-2823. 350. Rodrigues EG, Zavala F, Nussenzweig RS, Wilson JM, & Tsuji M (1998) Efficient induction of protective anti-malaria immunity by recombinant adenovirus. Vaccine 16(19):1812-1817. 351. Rodríguez A, et al. (2009) Evaluation of a prime-boost vaccine schedule with distinct adenovirus vectors against malaria in rhesus monkeys. Vaccine 27(44):6226-6233. 352. Stewart VA, et al. (2007) Priming with an Adenovirus 35-Circumsporozoite Protein (CS) Vaccine followed by RTS,S/AS01B Boosting Significantly Improves Immunogenicity to Plasmodium falciparum CS Compared to That with Either Malaria Vaccine Alone. Infect. Immun. 75(5):2283-2290. 353. Appledorn DM, et al. (2008) Wild-type adenoviruses from groups A-F evoke unique innate immune responses, of which HAd3 and SAd23 are partially complement dependent. Gene Ther 15(12):885-901. 354. Takeshita F, et al. (2006) Toll-Like Receptor Adaptor Molecules Enhance DNARaised Adaptive Immune Responses against Influenza and Tumors through Activation of Innate Immunity. J. Virol. 80(13):6218-6224. 260 355. Hedhli D, Dimier-Poisson I, Judge JW, Rosenberg B, & Mévélec MN (2009) Protective immunity against Toxoplasma challenge in mice by coadministration of T. gondii antigens and Eimeria profilin-like protein as an adjuvant. Vaccine 27(16):22742281. 356. Schuldt NJ, et al. (2011) Vaccine platforms combining circumsporozoite protein and potent immune modulators, rEA or EAT-2, paradoxically result in opposing immune responses. (Translated from eng) PLoS One 6(8):e24147 (in eng). 357. Dong Z & Veillette A (2010) How do SAP family deficiencies compromise immunity? Trends in Immunology 31(8):295-302. 358. Aldhamen YA, et al. (2011) Expression of the SLAM Family of Receptors Adapter EAT-2 as a Novel Strategy for Enhancing Beneficial Immune Responses to Vaccine Antigens. The Journal of Immunology 186(2):722-732. 359. Bruna-Romero O, Rocha CD, Tsuji M, & Gazzinelli RT (2004) Enhanced protective immunity against malaria by vaccination with a recombinant adenovirus encoding the circumsporozoite protein of Plasmodium lacking the GPI-anchoring motif. Vaccine 22(27-28):3575-3584. 360. Ophorst OJ, et al. (2006) Immunogenicity and protection of a recombinant human adenovirus serotype 35-based malaria vaccine against Plasmodium yoelii in mice. (Translated from eng) Infect Immun 74(1):313-320 (in eng). 361. Martins MA, et al. (2010) T-Cell Correlates of Vaccine Efficacy after a Heterologous Simian Immunodeficiency Virus Challenge. J. Virol. 84(9):4352-4365. 362. Yang Y, Huang C-T, Huang X, & Pardoll DM (2004) Persistent Toll-like receptor signals are required for reversal of regulatory T cell-mediated CD8 tolerance. Nat Immunol 5(5):508-515. 363. Appledorn DM, et al. (2010) A New Adenovirus Based Vaccine Vector Expressing an Eimeria tenella Derived TLR Agonist Improves Cellular Immune Responses to an Antigenic Target. PLoS ONE 5(3):e9579. 364. Lindsay RWB, et al. (2010) CD8+ T Cell Responses following Replication-Defective Adenovirus Serotype 5 Immunization Are Dependent on CD11c+ Dendritic Cells but Show Redundancy in Their Requirement of TLR and Nucleotide-Binding Oligomerization Domain-Like Receptor Signaling. J Immunol 185(3):1513-1521. 365. Appledorn DM, Patial S, Godbehere S, Parameswaran N, & Amalfitano A (2009) TRIF, and TRIF-interacting TLRs differentially modulate several adenovirus vectorinduced immune responses. (Translated from eng) J Innate Immun 1(4):376-388 (in eng). 261 366. Singh AP, et al. (2007) Plasmodium Circumsporozoite Protein Promotes the Development of the Liver Stages of the Parasite. Cell 131(3):492-504. 367. Aggarwal BB (2004) Nuclear factor-[kappa]B: The enemy within. Cancer Cell 6(3):203-208. 368. Torgler R, et al. (2008) Sporozoite-Mediated Hepatocyte Wounding Limits Plasmodium Parasite Development via MyD88-Mediated NF-{kappa}B Activation and Inducible NO Synthase Expression. J Immunol 180(6):3990-3999. 369. Shinoda K, et al. (2010) Regulation of human dendritic cells by a novel specific nuclear factor-[kappa]B inhibitor, dehydroxymethylepoxyquinomicin. Human Immunology 71(8):763-770. 370. Hartman ZC, Black EP, & Amalfitano A (2007) Adenoviral infection induces a multifaceted innate cellular immune response that is mediated by the toll-like receptor pathway in A549 cells. Virology 358(2):357-372. 371. Arun Kumar K, et al. (2006) The circumsporozoite protein is an immunodominant protective antigen in irradiated sporozoites. Nature 444(7121):937-940. 372. Grüner AC, et al. (2007) Sterile Protection against Malaria Is Independent of Immune Responses to the Circumsporozoite Protein. PLoS ONE 2(12):e1371. 373. Bleharski JR, Niazi KR, Sieling PA, Cheng G, & Modlin RL (2001) Signaling Lymphocytic Activation Molecule Is Expressed on CD40 Ligand-Activated Dendritic Cells and Directly Augments Production of Inflammatory Cytokines. J Immunol 167(6):3174-3181. 374. Ostrakhovitch EA, Wang Y, & Li SSC (2009) SAP binds to CD22 and regulates B cell inhibitory signaling and calcium flux. Cellular Signalling 21(4):540-550. 375. Li C, et al. (2008) The X-linked lymphoproliferative syndrome gene product SAP regulates B cell function through the Fc[gamma]RIIB receptor. Cellular Signalling 20(11):1960-1967. 376. Detre C, Keszei M, Romero X, Tsokos G, & Terhorst C (2010) SLAM family receptors and the SLAM-associated protein (SAP) modulate T cell functions. Seminars in Immunopathology 32(2):157-171. 377. Schmidt NW, Butler NS, Badovinac VP, & Harty JT (2010) Extreme CD8 T Cell Requirements for Anti-Malarial Liver-Stage Immunity following Immunization with Radiation Attenuated Sporozoites. PLoS Pathog 6(7):e1000998. 378. Kiepiela P, et al. (2007) CD8+ T-cell responses to different HIV proteins have discordant associations with viral load. Nat Med 13(1):46-53. 262 379. Doolan DL & Hoffman SL (2000) The Complexity of Protective Immunity Against Liver-Stage Malaria. J Immunol 165(3):1453-1462. 380. Liu MA (Immunologic basis of vaccine vectors. (Translated from eng) Immunity 33(4):504-515 (in eng). 381. Coffman RL, Sher A, & Seder RA (Vaccine adjuvants: putting innate immunity to work. (Translated from eng) Immunity 33(4):492-503 (in eng). 382. Williamson ED & Titball RW (2002) Vaccines against dangerous pathogens. (Translated from eng) Br Med Bull 62:163-173 (in eng). 383. Mbow ML, De Gregorio E, Valiante NM, & Rappuoli R (New adjuvants for human vaccines. (Translated from eng) Curr Opin Immunol 22(3):411-416 (in eng). 384. Hawlisch H & Kohl J (2006) Complement and Toll-like receptors: key regulators of adaptive immune responses. (Translated from eng) Molecular immunology 43(12):13-21 (in eng). 385. Iwasaki A & Medzhitov R (2004) Toll-like receptor control of the adaptive immune responses. (Translated from eng) Nature immunology 5(10):987-995 (in eng). 386. Rosenberg B, et al. (2005) Protein from intestinal Eimeria protozoan stimulates IL12 release from dendritic cells, exhibits antitumor properties in vivo and is correlated with low intestinal tumorigenicity. (Translated from eng) International journal of cancer 114(5):756-765 (in eng). 387. Blanco P, Palucka AK, Pascual V, & Banchereau J (2008) Dendritic cells and cytokines in human inflammatory and autoimmune diseases. (Translated from eng) Cytokine & growth factor reviews 19(1):41-52 (in eng). 388. Morefield GL, et al. (2005) Role of aluminum-containing adjuvants in antigen internalization by dendritic cells in vitro. (Translated from eng) Vaccine 23(13):1588-1595 (in eng). 389. Tritto E, Mosca F, & De Gregorio E (2009) Mechanism of action of licensed vaccine adjuvants. (Translated from eng) Vaccine 27(25-26):3331-3334 (in eng). 390. Lambrecht BN, Kool M, Willart MA, & Hammad H (2009) Mechanism of action of clinically approved adjuvants. (Translated from eng) Curr Opin Immunol 21(1):2329 (in eng). 391. Laurent F, Bourdieu C, Kazanji M, Yvore P, & Pery P (1994) The immunodominant Eimeria acervulina sporozoite antigen previously described as p160/p240 is a 19kilodalton antigen present in several Eimeria species. (Translated from eng) Mol Biochem Parasitol 63(1):79-86 (in eng). 263 392. Jenkins MC, Lillehoj HS, & Dame JB (1988) Eimeria acervulina: DNA cloning and characterization of recombinant sporozoite and merozoite antigens. (Translated from eng) Exp Parasitol 66(1):96-107 (in eng). 393. Boonstra A, et al. (2006) Macrophages and myeloid dendritic cells, but not plasmacytoid dendritic cells, produce IL-10 in response to MyD88- and TRIFdependent TLR signals, and TLR-independent signals. (Translated from eng) J Immunol 177(11):7551-7558 (in eng). 394. Yamamoto M, et al. (2003) Role of adaptor TRIF in the MyD88-independent toll-like receptor signaling pathway. (Translated from eng) Science 301(5633):640-643 (in eng). 395. Lee MS & Kim YJ (2007) Signaling pathways downstream of pattern-recognition receptors and their cross talk. (Translated from eng) Annu Rev Biochem 76:447-480 (in eng). 396. Power MR, Li B, Yamamoto M, Akira S, & Lin TJ (2007) A role of Toll-IL-1 receptor domain-containing adaptor-inducing IFN-beta in the host response to Pseudomonas aeruginosa lung infection in mice. (Translated from eng) J Immunol 178(5):31703176 (in eng). 397. Kumar H, Koyama S, Ishii KJ, Kawai T, & Akira S (2008) Cutting edge: cooperation of IPS-1- and TRIF-dependent pathways in poly IC-enhanced antibody production and cytotoxic T cell responses. (Translated from eng) J Immunol 180(2):683-687 (in eng). 398. McAleer JP, Rossi RJ, & Vella AT (2009) Lipopolysaccharide potentiates effector T cell accumulation into nonlymphoid tissues through TRIF. (Translated from eng) J Immunol 182(9):5322-5330 (in eng). 399. Hoebe K, et al. (2003) Upregulation of costimulatory molecules induced by lipopolysaccharide and double-stranded RNA occurs by Trif-dependent and Trifindependent pathways. (Translated from eng) Nature immunology 4(12):1223-1229 (in eng). 400. Appledorn DM, Patial S, Godbehere S, Parameswaran N, & Amalfitano A (2009) TRIF, and TRIF-Interacting TLRs Differentially Modulate Several Adenovirus VectorInduced Immune Responses. J Inn Imm 1:376-388. 401. Choi YJ, Im E, Pothoulakis C, & Rhee SH (TRIF modulates TLR5-dependent responses by inducing proteolytic degradation of TLR5. (Translated from eng) J Biol Chem 285(28):21382-21390 (in eng). 402. Kenny EF & O'Neill LA (2008) Signalling adaptors used by Toll-like receptors: an update. (Translated from eng) Cytokine 43(3):342-349 (in eng). 264 403. Biswas SK, et al. (2007) Role for MyD88-independent, TRIF pathway in lipid A/TLR4-induced endotoxin tolerance. (Translated from eng) J Immunol 179(6):4083-4092 (in eng). 404. Kawai T & Akira S (2007) TLR signaling. (Translated from eng) Semin Immunol 19(1):24-32 (in eng). 405. Kumar H, Kawai T, & Akira S (2009) Toll-like receptors and innate immunity. (Translated from eng) Biochem Biophys Res Commun 388(4):621-625 (in eng). 406. Kantoff PW, et al. (Sipuleucel-T immunotherapy for castration-resistant prostate cancer. (Translated from eng) The New England journal of medicine 363(5):411-422 (in eng). 407. Conroy H, Marshall NA, & Mills KH (2008) TLR ligand suppression or enhancement of Treg cells? A double-edged sword in immunity to tumours. (Translated from eng) Oncogene 27(2):168-180 (in eng). 408. Kawai T & Akira S (2008) Toll-like receptor and RIG-I-like receptor signaling. (Translated from eng) Ann N Y Acad Sci 1143:1-20 (in eng). 409. Appledorn DM, Aldhamen YA, Godbehere S, Seregin SS, & Amalfitano A (2011) Sublingual administration of an adenovirus serotype 5 (Ad5)-based vaccine confirms Toll-like receptor agonist activity in the oral cavity and elicits improved mucosal and systemic cell-mediated responses against HIV antigens despite preexisting Ad5 immunity. (Translated from eng) Clin Vaccine Immunol 18(1):150160 (in eng). 410. Veillette A (2010) SLAM-family receptors: immune regulators with or without SAPfamily adaptors. (Translated from eng) Cold Spring Harb Perspect Biol 2(3):a002469 (in eng). 411. Stark S & Watzl C (2006) 2B4 (CD244), NTB-A and CRACC (CS1) stimulate cytotoxicity but no proliferation in human NK cells. (Translated from eng) Int Immunol 18(2):241-247 (in eng). 412. Kumaresan PR, Lai WC, Chuang SS, Bennett M, & Mathew PA (2002) CS1, a novel member of the CD2 family, is homophilic and regulates NK cell function. (Translated from eng) Mol Immunol 39(1-2):1-8 (in eng). 413. Bloch-Queyrat C, et al. (2005) Regulation of natural cytotoxicity by the adaptor SAP and the Src-related kinase Fyn. (Translated from eng) The Journal of experimental medicine 202(1):181-192 (in eng). 265 414. Berger SB, et al. (SLAM is a microbial sensor that regulates bacterial phagosome functions in macrophages. (Translated from eng) Nat Immunol 11(10):920-927 (in eng). 415. Aldhamen YA, et al. (Expression of the SLAM Family of Receptors Adapter EAT-2 as a Novel Strategy for Enhancing Beneficial Immune Responses to Vaccine Antigens. (Translated from eng) J Immunol 186(2):722-732 (in eng). 416. Veillette A (SLAM-family receptors: immune regulators with or without SAP-family adaptors. (Translated from eng) Cold Spring Harb Perspect Biol 2(3):a002469 (in eng). 417. Bhat R, Eissmann P, Endt J, Hoffmann S, & Watzl C (2006) Fine-tuning of immune responses by SLAM-related receptors. (Translated from eng) J Leukoc Biol 79(3):417-424 (in eng). 418. Zhong MC & Veillette A (2008) Control of T lymphocyte signaling by Ly108, a signaling lymphocytic activation molecule family receptor implicated in autoimmunity. (Translated from eng) J Biol Chem 283(28):19255-19264 (in eng). 419. Morra M, et al. (2001) Characterization of SH2D1A missense mutations identified in X-linked lymphoproliferative disease patients. (Translated from eng) J Biol Chem 276(39):36809-36816 (in eng). 420. Clarkson NG, Simmonds SJ, Puklavec MJ, & Brown MH (2007) Direct and indirect interactions of the cytoplasmic region of CD244 (2B4) in mice and humans with FYN kinase. (Translated from eng) J Biol Chem 282(35):25385-25394 (in eng). 421. Appledorn DM, et al. (2008) Adenovirus vector-induced innate inflammatory mediators, MAPK signaling, as well as adaptive immune responses are dependent upon both TLR2 and TLR9 in vivo. (Translated from eng) J Immunol 181(3):21342144 (in eng). 422. Calpe S, et al. (2008) The SLAM and SAP gene families control innate and adaptive immune responses. (Translated from eng) Adv Immunol 97:177-250 (in eng). 423. Boles KS & Mathew PA (2001) Molecular cloning of CS1, a novel human natural killer cell receptor belonging to the CD2 subset of the immunoglobulin superfamily. (Translated from eng) Immunogenetics 52(3-4):302-307 (in eng). 424. Bouchon A, Cella M, Grierson HL, Cohen JI, & Colonna M (2001) Activation of NK cellmediated cytotoxicity by a SAP-independent receptor of the CD2 family. (Translated from eng) Journal of immunology 167(10):5517-5521 (in eng). 266 425. Zhu J, Huang X, & Yang Y (2010) NKG2D is required for NK cell activation and function in response to E1-deleted adenovirus. (Translated from eng) J Immunol 185(12):7480-7486 (in eng). 426. Sintes J, Romero X, de Salort J, Terhorst C, & Engel P (2010) Mouse CD84 is a panleukocyte cell-surface molecule that modulates LPS-induced cytokine secretion by macrophages. (Translated from eng) J Leukoc Biol 88(4):687-697 (in eng). 427. Yarilina A, Park-Min KH, Antoniv T, Hu X, & Ivashkiv LB (2008) TNF activates an IRF1-dependent autocrine loop leading to sustained expression of chemokines and STAT1-dependent type I interferon-response genes. (Translated from eng) Nature immunology 9(4):378-387 (in eng). 428. Ward RC & Kaufman HL (2007) Targeting costimulatory pathways for tumor immunotherapy. (Translated from eng) Int Rev Immunol 26(3-4):161-196 (in eng). He TC, et al. (1998) A simplified system for generating recombinant adenoviruses. (Translated from eng) Proceedings of the National Academy of Sciences of the United States of America 95(5):2509-2514 (in eng). 429. 430. Cotter MJ, Zaiss AK, & Muruve DA (2005) Neutrophils interact with adenovirus vectors via Fc receptors and complement receptor 1. (Translated from eng) J Virol 79(23):14622-14631 (in eng). 431. Ng P & Graham FL (2002) Construction of first-generation adenoviral vectors. (Translated from eng) Methods in molecular medicine 69:389-414 (in eng). 432. Rader JS, et al. (2008) Phase I study and preliminary pharmacology of the novel innate immune modulator rBBX-01 in gynecologic cancers. (Translated from eng) Clin Cancer Res 14(10):3089-3097 (in eng). 433. Tatsis N & Ertl HC (2004) Adenoviruses as vaccine vectors. (Translated from eng) Mol Ther 10(4):616-629 (in eng). 434. Weaver EA, et al. (2009) Comparison of replication-competent, first generation, and helper-dependent adenoviral vaccines. (Translated from eng) PLoS One 4(3):e5059 (in eng). 267